Tetralogy of Fallot

Poster 93A all pigs. Myocardial perfusion in the normally-perfused LAD and collateralperfused LCx regions were determined with colored mlcrospherus. Coronary micrevassals were etudied in vitro with video-microscopy. Responses = % relaxation of acetylcholine-induced contraction. Group AOP (10/~M) PlnacidJl (10 #M) SNP (10/~M) LAD-control 70 ± 3* 83 ± 1 73 ± 4 LCx-¢ontml 46±4 82±4 76±3 LAD-VEGF 77±7" 84±4 82±6 LCx-VEGF 75 ± 3* 92 ± 4 70 ± 5 *p < 0.05 vs LCx-conlrol. Relaxations to pinacidil (K + ATP channel opener) and nitroprusside (SNP) were similar in all groups. Endethellum-depandent relaxation to ADP was impetred by collateral-dependent perfusion. These responses were significantly preserved with VEGF treatment. LCx/LAD blood flow ratio at rest was similar in the VEGF (1.10) and control (1.15) groups, but dudng atrial pacing, perfusion was improved in the VEGF-treated animals (1.50) compamcl with control pigs (0.98, p < 0.01 vs VEGF). The improved vascular function and pertusion associated with VEGF treatment may have implications regarding the management of patients with severe coronary disease who are not candidates for conventional methods of revasculadzetion.

The management of Tetralogy of Fallot with concomitant pulmonary tuberculosis poses significant challenges, especially regarding the optimal timing for cardiac surgery. Currently, no consensus or algorithmic guidelines exist regarding screening for pulmonary tuberculosis in this cohort of CHD patients or the appropriate waiting period before proceeding with surgery. Patients may have to wait until full resolution of pulmonary tuberculosis before surgery is conducted. We present a case of a 20 year old female presenting with pulmonary tuberculosis complicating Tetralogy of Fallot who successfully underwent surgery after 2 months of intensive phase TB therapy with Isoniazid, Rifampicin, Pyrazinamide and Ethambutol.

Serious anaphylactic reactions to anesthetics can be life-threatening events. Vecuronium is promoted as a neuromuscular blocking drug that is least likely to cause anaphylaxis due to its low histamine-releasing potential. Perioperative hypoxemia in patients with tetralogy of Fallot (TOF) due to anaphylactic reactions can be easily misdiagnosed as anoxic spells. We report a patient with TOF who suffered from symmetrical peripheral gangrene after undergoing catecholamine-refractory anaphylactic shock to vecuronium perioperatively. CPB (cardiopulmonary bypass) is the most effective support for severe anaphylactic shock during the perioperative period. Intradermal test screening for anaphylaxis to anesthetics is an effective method for prevention and diagnosis.

A 33-year-old male patient with uncorrected tetralogy of Fallot was hospitalised for multiple peripheral arterial emboli. Bilateral above-knee amputation had been done after unsuccessful femoral embolectomy. A large thrombus was detected in the apical portion of the left ventricle which was the source of the embolus.The patient complained of mild frontal headache and progressive right-sided weakness shortly after an echocardiographic examination. A computed tomography (CT) scan revealed a left middle cerebral artery territory infarct. Patients with grown-up cyanotic congenital heart disease are at increased risk of thromboembolic cerebrovascular events. This report highlights the necessity for physicians to be alert for uncommon causes of acute stroke.

Congenital anomalies of the heart and cardiovascular system occur in 7-10 per 1000 live births (0.7-1%). It is the commonest form of congenital disease and accounts for approximately 30% of the total burden of congenital disease. With a decline in the incidence of rheumatic heart disease, congenital heart disease has become the main cause of heart disease in children in the developed world; 10-15% of affected children have associated congenital anomalies of the skeletal, genitourinary or gastrointestinal systems. Approximately 10-15% of patients with congenital heart disease may survive untreated to adulthood but the majority require some form of cardiac surgery as children. Nine lesions comprise more than 80% of congenital heart disease while a wide range of more unusual and complex lesions comprise the remainder. Surgical closure of a patent ductus arteriosus was first performed in 1939. In 1945 the first surgical repair of coarctation of the aorta was reported 27 54 and the Blalock-Taussig shunt from the left subclavian to the left pulmonary artery was introduced as a palliative procedure for tetralogy of Fallot 12 allowing patients with this lesion to live for several decades. In 1955 a series of patients who underwent successful open heart surgery with cardiopulmonary bypass to correct septal defects was reported. Advances in cardiological diagnosis and management, surgical techniques, anaesthesia and intensive care have resulted in a situation where the majority of conditions are operated up on with a high proportion (Br.

Pacemaker implantation in congenitally corrected transposition of the great arteries can be challenging due to distorted cardiac anatomy that in some cases it may be accompanied by other anomalies. The present report outlines a rare case of congenitally corrected transposition of the great arteries, asymptomatic until the seventh decade of life.

Case Scenario: 1 A full term born, 6 month old child presented in outpatient department with chief complaints of respiratory distress, poor weight gain and recurrent chest infections since birth. On general examination, PR was 152/min, BP-80/48 mm Hg. Cardiac examination revealed an ejection systolic murmur at left lower sternal border, S2 split with accentuation of pulmonary component and apical diastolic rumble. ECG showed features of left atrial enlargement and and left ventricular hypertrophy. Chest x-ray showed cardiomegaly and increased pulmonary vascular marking. Echocardiography revealed 12 mm ventricular septal defect with significant left to right shunt, aortic cusp prolapse and mild aortic insufficiency. Q 1: What is the most appropriate line of treatment in this patient? a. Medical Management b. Surgical Treatment c. Percutaneous Device closure d. All of the above Q 2: What is the cause of apical rumble in this patient? a. Increased flow through mitral valve b. Left vent...

Aim: Although limited in assessing right ventricular (RV) function, echocardiography is widely used after correction for tetralogy of Fallot (TOF). The change in echocardiographic measurements of RV in asymptomatic patients after TOF repair over a long follow-up time is not explored yet. The variation in simple echocardiographic measurements during follow-up of our TOF patients are presented. The predictive value of those parameters in determining a future pulmonary valve replacement is sought. Method: Asymptomatic patients surviving the first year after correction for simple TOF from February 2007 to December 2019 at Başkent University, Istanbul Hospital are enrolled. Patients are followed annually with echocardiography including: RV area, volume, length, RV outflow tract (RVOT) diameter and gradient, tricuspid annulus diameter, tricuspid lateral annular tissue velocities, tricuspid annular plane systolic excursion, TEI index RV ejection fraction (EF) measurements. The change in th...

Objectives: To describe a rare case of chemodectoma, its clinical features and management, and to discuss its relationship chronic hypoxia from Tetralogy of Fallot. Methods: Design: Case Report Setting: Tertiary Government Hospital Patient: One Result: A 23-year-old woman presented with a painless, slow growing, movable right submandibular mass, initially diagnosed as a lipoma by fine needle aspiration biopsy.  Computed tomography scan showed a solid nodule with ill-defined margins from the angle of the mandible to the level of the hyoid bone along the carotid sheath. There was also an incidental finding of patent ductus arteriosus and Tetralogy of Fallot on pre-operative clearance. Excision of the mass under general anesthesia revealed adherence to the posterior portion of the carotid trunk enveloping both the internal & external carotid artery. Final histopathological diagnosis was chemodectoma. Conclusion: Although rare, chemodectoma should be considered as one of the differentia...

This position paper addresses the critical need for standardized clinical protocols to manage elevated hematocrit (polycythemia) in patients receiving long-term Antiretroviral Therapy (ART). While ART has successfully transformed HIV into a chronic condition, the elevated hematocrit seen in a subset of patients often driven by factors like testosterone replacement therapy (TRT), chronic hypoxia, or comorbidities significantly increases blood viscosity. This state of hyperviscosity compounds the pre-existing pro-thrombotic risk inherent to chronic HIV inflammation, leading to a heightened danger of severe thrombotic events, including stroke and myocardial infarction (Triant & Lee, 2014; Vorkas et al., 2011). Our position is that routine, riskstratified monitoring of hematocrit is mandatory. We recommend a proactive management strategy encompassing etiology investigation (differentiating secondary polycythemia from Polycythemia Vera), non-ART interventions like TRT dose adjustment and smoking cessation, and the therapeutic use of phlebotomy to maintain safe hematocrit targets (Hillis, 2020). Implementing these measures is essential to mitigate cardiovascular morbidity, ensuring the sustained long-term safety and quality of life for individuals on ART.

Geometric ring annuloplasty is a recent adjunct in valve repair for aortic regurgitation (AR). This concept has spar- ingly been applied to congenital aortic or neoaortic valve dysfunction. Root dilatation is common in tetralogy of Fallot (TOF), particularly in adulthood late after repair, although severe AR and other complications are less frequent. We present the technical details of aortic valve repair comprising geometric prosthetic ring implantation in an adult patient with surgically corrected TOF.

Two pairs of twins with specific concordance in congenital heart lesions are presented. We reviewed all the monozygotic twins with specific concordance in congenital heart lesions in the last 18 years. Two pairs were found. First, a pair of 13 years-old monozygotic female twins; a severe infundibular and pulmonary valve stenosis was discovered in both girls. In the second pair of twins (8 years-old boys), Tetralogy of Fallot was diagnosed by echocardiogram, cardiac catheterization and angicardiography. In fluorescence hybridization test in situ all patients were negative for microdeletion in chromosome 22q11. Anatomic differences between twins are not enough explained on genetic bases; it&#39;s necessary to consider the role of other factors, probably acquired during the first stages of embryonic development. These are the two first pairs of twins with specific concordance in congenital cardiac lesions reported in Mexico.

Tetralogy of Fallot is the most common cyanotic congenital cardiac diseases. It is also associated with other cardiac abnormalities; however, its association with dextrocardia and situs inversus is rarely reported. We report a case of these findings in a 9-year-old boy, describe the patient's postoperative course, and review the medical literature relevant to these combined conditions.

Background Epidemiological studies indicate a substantial excess familial recurrence of non-syndromic Tetralogy of Fallot (TOF), implicating genetic factors that remain largely unknown. The Rho induced kinase 1 gene (ROCK1) is a key component of the planar cell polarity signalling pathway, which plays an important role in normal cardiac development. The aim of this study was to investigate the role of genetic variation in ROCK1 on the risk of TOF. Results ROCK1 was sequenced in a discovery cohort of 93 non-syndromic TOF probands to identify rare variants. TagSNPs were selected to capture commoner variation in ROCK1. Novel variants and TagSNPs were genotyped in a discovery cohort of 458 TOF cases and 1331 healthy controls, and positive findings were replicated in a further 209 TOF cases and 1290 healthy controls. Association between genotypes and TOF was assessed using LAMP. A rare SNP (c.807C &gt; T; rs56085230) discovered by sequencing was associated with TOF risk (p = 0.006) in th...

While manifold learning from images itself has become widely used in medical image analysis, the accuracy of existing implementations suffers from viewing each image as a single data point. To address this issue, we parcellate images into regions and then separately learn the manifold for each region. We use the regional manifolds as low-dimensional descriptors of high-dimensional morphological image features, which are then fed into a classifier to identify regions affected by disease. We produce a single ensemble decision for each scan by the weighted combination of these regional classification results. Each weight is determined by the regional accuracy of detecting the disease. When applied to cardiac magnetic resonance imaging of 50 normal controls and 50 patients with reconstructive surgery of Tetralogy of Fallot, our method achieves significantly better classification accuracy than approaches learning a single manifold across the entire image domain.

Follow-up studies on patients operated on for congenital heart defects have shown good anatomical results and long-term survival. To date, there have been few studies on such patients with regard to long-term psychosocial outcome and quality of life. In this study, two cohorts of patients operated on before the age of 15 years, one for tetralogy of Fallot (TOF) and the other for atrial septal defect (ASD), were investigated 20 and 30 years after operation regarding quality of life. The combined cohort had a higher educational level than average. There was no connection between quality of life and physical health as judged from the New York Heart Association classification. The TOF group rated their quality of life higher than the ASD group, but both groups had lower figures at the 30-year than at the 20-year follow-up. Fewer patients in the TOF than in the ASD group considered that their lives were affected by the heart disease. It is concluded that the severity of the heart disease is not necessarily congruent with estimated quality of live and that mild heart defects, such as ASD, can have a considerable impact on later life quality. Surprisingly few TOF patients were affected negatively, a finding which might reflect development of a specific coping strategy in these patients during childhood.

Seorang anak perempuan, 5 tahun mengalami blok AV total pasca operasi VSD perimembranous besar sehingga direncanakan pemasangan pacu jantung permanen. Pada saat punksi vena subklavia kanan wire peelaway sheath tidak dapat masuk ke atrium kanan. Injeksi kontras melalui abocath memperlihatkan oklusi vena cava superior kemungkinan oleh thrombus yang terorganisasi. Samar-samar terlihat kontras mengisi vena kana superior kiri persisten melalui kolateral dari vena kava superior kanan (Gambar 1). Maka diputuskan untuk melakukan pemasangan pacu jantung menetap (PJM) melalui vena subklavia kiri ke alur keluar ventrikel kanan (RVOT= right ventricle outflow tract) melalui vena kava superior kiri persisten.

Background. Balloon Mitral Valvuloplasty (BMV) can provides effectively the mechanical obstruction relief in mitral stenosis. Achieving a successful BMV based on echocardiographic criteria potentially increase mitral regurgitation complication. In addition, long-term clinical outcome of a successful BMV based on catheterization criteria has not been widely elaborated. This study aims to compare the long-term clinical outcome of BMV based on the echocardiographic criteria and the catheterization criteria. Methods. A cross sectional study was conducted in mitral stenosis patients after BMV procedure. Patients was divided into 2 groups based on successful echocardiographic criteria (MVA ≥ 1,5 cm2 vs MVA &lt; 1,5 cm2). The long-term clinical outcome was evaluated using 6 minute walk test and SF-36 questionnaire. Results. Twenty two female patients were fulfilling study criteria with aged 38,8 ±12,0 years and 63,6% in atrial fibrillation. The characteristic between two groups were not si...

Tachycardia of right ventricular outflow tract (RVOT) is the most common cause of nonischemic ventricular tachycardia (VT). It is commonly due to idiopathic RVOT tachycardia. Idiopathic RVOT tachycardia is a nonfamilial and benign condition that occurs in young individuals without structural heart disease. Radiofrequency ablation has a high successful rate to eliminate this arrhythmia. Non contact mapping can be very helpful in patient with nonsustained tachycardias. We report the case of a 55-year-old man who presented with non sustained ventricular tachycardia and was successfully underwent radiofrequency ablation using non contact mapping.

Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness. Methods: In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise. Results: In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic c...

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an acquired immune-mediated neuropathy
characterized by progressive weakness and sensory dysfunction. This case describes a 43-year-old male with
CIDP, complicated by diabetes, hypertension, ischemic heart disease, and MRSA positivity. Physiotherapy
interventions emphasized positioning, range of motion, trunk facilitation, and modified respiratory care under strict
infection-control precautions. After two weeks of therapy, the patient demonstrated preserved joint range, reduced
edema, improved sitting tolerance, and prevention of secondary respiratory complications. This case highlights
the importance of adaptable physiotherapy strategies in neurological patients with concurrent infection risks.

Tetralogy of Fallot is the most commonly seen cyanotic congenital heart disease. It includes four cardinal features: right ventricular outflow tract obstruction, misaligned ventricular septal defect, overriding aorta, and concentric right ventricular hypertrophy. Intracranial pathologies such as brain abscesses, cerebrovascular accidents, and intracranial haemorrhage all have been reported with Tetralogy of Fallot. Here we present a case who had subarachnoid haemorrhage with vitreous haemorrhage in Tetralogy of Fallot.

Congenital heart disease (CHD) is the most frequently occurring congenital disorder responsible for 28% (1/3 rd ) of all congenital birth defects. Its estimated incidence is up to 6-8 in 1000 live births and 45% of deaths caused by congenital defects include cardiac malformations. Clinical spectrum of CHD is versatile and varies with the type of CHD ranging from poor sucking, cyanosis, respiratory distress, failure to thrive to frank heart failure. Asymptomatic presentation is common and discovered accidentally during routine check-up visits and requiring subsequent ECHO evaluation. However, role of chest x-ray and ECG in complementing the diagnosis of CHD should not be underestimated. 5 There have been many previous studies in Odisha showing prevalence, clinical features, diagnosis of CHD but very few studies show clinico-echo correlation in CHD patients. Our hospital is prime tertiary care teaching hospital not only for southern Odisha but also for neighbouring state like Andhra Pradesh. So, we wanted to know what is the diagnostic reliability of clinical ABSTRACT Background: Congenital heart disease (CHD) is the most frequently occurring congenital disorder responsible for 1/3rd of all congenital birth defects. Diagnostic accuracy of CHD is crucial for timely intervention and optimal patient outcome. So, the study has been done to look for the clinical profile and establishing correlation of clinical diagnosis with echocardiography diagnosis. Methods: This is a prospective cross-sectional study conducted in the department of pediatrics MKCG MCH, Berhampur over a period of 2 years. All children between 1 month to 14 years admitted for suspected CHD were included in the study. Clinical diagnosis was made based on detailed history and physical examination supported by chest X-ray and ECG. Final diagnosis was confirmed by echocardiography and clinico-ECHO correlation was deduced. The 188 patients with CHD were included in this study. The most common age of presentation was 1 month to 1 year with 71.2%, male to female ratio was 1.06:1. Acyanotic CHD (ACHD) was found to be 70.29%, while 29.7% had congenital cyanotic heart disease (CCHD). Clinically specific diagnosis was attributed to 67.1% of cases and no specific diagnosis could be made in 32.9%. Isolated CHD was found to be 66.4% and Complex CHD with multiple defects was found to be 33.6%. Clinic echo correlation was accurate in 39.4% cases, partially correlated in 32% and no correlation in 28.6% cases. Conclusions: So, echocardiography remains the gold standard for diagnosis of CHD and guiding the treatment. Clinical diagnosis alone may not detect associated CHD.

Background Epidemiological studies indicate a substantial excess familial recurrence of non-syndromic Tetralogy of Fallot (TOF), implicating genetic factors that remain largely unknown. The Rho induced kinase 1 gene (ROCK1) is a key component of the planar cell polarity signalling pathway, which plays an important role in normal cardiac development. The aim of this study was to investigate the role of genetic variation in ROCK1 on the risk of TOF. Results ROCK1 was sequenced in a discovery cohort of 93 non-syndromic TOF probands to identify rare variants. TagSNPs were selected to capture commoner variation in ROCK1. Novel variants and TagSNPs were genotyped in a discovery cohort of 458 TOF cases and 1331 healthy controls, and positive findings were replicated in a further 209 TOF cases and 1290 healthy controls. Association between genotypes and TOF was assessed using LAMP. A rare SNP (c.807C &gt; T; rs56085230) discovered by sequencing was associated with TOF risk (p = 0.006) in th...

Patients with Down&#39;s syndrome are prone to have congenital heart defects. This study was conducted to evaluate the frequency of various congenital heart defects in children with Down&#39;s syndrome. The data was collected from the Department of Cardiology in FIC (Faisalabad Institute of Cardiology) Hospital. Fifty-eight phenotypically Down syndrome children coming to the cardiology department for echocardiography, from birth to 13 years were included in this study. After detailed history and physical examination, all these patients were subjected to 2-dimentional echocardiography in addition to routine laboratory investigations. Congenital heart defects were found in 29 out of 58 patients (50%). Among the affected patients, 16 (55.2%) were males and 13 (44.8%) females with male to female ratio of 1.2:1. Acynotic lesions were more common (79.31%) than cyanotic lesion (20.69%). Among the isolated lesions ventricular septal defect, patent ductus arteriosus and complete atrioventric...

OBJECTIVES: Since 1996, our centre performs restrictive enlargement of the pulmonary annulus at surgical repair of tetralogy of Fallot. A transannular patch is only used if the z-score of the pulmonary annulus is smaller than -2. We sought to determine whether this strategy reduces pulmonary insufficiency (PI) and reoperation rate compared to a nationwide contemporary cohort that has not been operated using a uniform strategy. METHODS: Eighty-seven tetralogy of Fallot patients were included in the study (Group 1). One hundred sixty-seven tetralogy of Fallot patients from the Competence Network for Congenital Heart Disease served as controls (Group 2). Clinical, echocardiographic, electrocardiogram, cardiovascular magnetic resonance and outcome data were analysed. RESULTS: Follow-up time since repair was not different between groups [12.9 (7.8-18.8) vs 13.1 (5.2-16.9) years, P = 0.96] while transannular patch rate was significantly lower in Group 1 (32.2% vs 64.7%, P < 0.001). Ten-year freedom from reoperation for PI was significantly higher in our cohort (98% vs 92%, P = 0.01). Multivariable analysis identified restrictive enlargement as the only predictor for no need of reoperation [hazard ratio 0.4 (95% confidence interval 0.24-0.84), P < 0.01]. In Group 1, cardiovascular magnetic resonance-derived volumes were smaller (indexed end-diastolic volume: 103 ± 24 vs 123 ± 31 ml/m 2 , P < 0.001; indexed end-systolic volume: 53 ± 19 vs 59 ± 20 ml/m 2 , P = 0.04) and regurgitation fraction was lower (21 ± 14 vs 31 ± 17%, P < 0.001). Restrictive enlargement of the pulmonary annulus reduces PI and limits the amount of right ventricular dilatation at intermediate-term follow-up. The need for reoperation to alleviate PI is significantly lower compared to a contemporary cohort. Restrictive enlargement strategy has a stronger influence on freedom from reoperation than the use of a transannular patch.

Objectives: Retrospective data suggest that a wide pulmonary annulus after Fallot repair aggravates pulmonary regurgitation. Therefore, since 1997, in our institution transannular patch enlargement was only intended for patients with a native pulmonary annulus z-score less than À4. If transannular patching was needed, enlargement was aimed to diameters within the range of a z-score of À2. We sought to determine whether this strategy of restrictive enlargement of the pulmonary annulus was adequate to reduce transannular patch rate and to limit pulmonary annulus width without increased right ventricular pressure load. Methods: Two-hundred-and-sixteen Fallot patients were retrospectively analysed. Ninety-eight patients underwent repair between 1997 and 2006 adhering to our uniform strategy (Group 1). One hundred and eighteen patients were operated between 1977 and 1996 without a uniform strategy (Group 2). Transannular patch rate, native and postoperative pulmonary annulus z-score, postoperative right ventricular outflow tract velocity on echocardiography and early reoperation rate for right ventricular outflow tract obstruction were analysed in both groups. Results: Compared to Group 2, patients in Group 1 were younger at repair, transannular patch rate was significantly reduced (32 vs 68%, p < 0.0001) and postoperative pulmonary annulus diameters were smaller (z-score À2.1 AE 1.5 vs 0.0 AE 3.1, p < 0.0001). However, no difference in right ventricular outflow tract velocity (2.4 AE 0.8 vs 2.2 AE 0.8 m/s; p = NS) or the incidence of early reoperation for right ventricular outflow tract obstruction was found between the groups (3/98 vs 1/118; p = NS). Conclusion: Restrictive enlargement of the pulmonary annulus at Fallot repair lowers transannular patch rate, limits the postoperative width of the pulmonary annulus but does not result in increased right ventricular pressure load or reoperation rate for residual right ventricular outflow tract obstruction. A limitation of postoperative pulmonary regurgitation can be expected when the extent of pulmonary annulus enlargement at repair is limited.

Surgical repair of tetralogy of Fallot is associated with low early morbidity and mortality. However, there may be late morbidity and mortality due to right ventricular dysfunction. The transatrial/transpulmonary technique may ameliorate these long-term complications. Here we present the results from our use of this approach. A hundred sixty-three consecutive patients (age 6 months to 45 years, median 1.5 years) underwent transatrial/transpulmonary total correction in our department. In 142 patients the main pulmonary artery was augmented by an autologous pericardial patch, in 31 cases the arterioplasty was extended to the pulmonary artery branches, and pulmonary artery valvuloplasty was needed in 129 patients. A monocusp autologous pericardial valve mechanism was inserted in 14 patients. Patient follow up was 100% complete with a median duration of 3.05 years. There were no deaths. One patient required early reoperation to relieve residual right ventricular outflow tract (RVOT) obs...

Background Reliable, non-invasive evaluation of right ventricular function, especially in congenital heart disease, is challenging. Objectives The aim of this study was to evaluate Doppler tissue imaging (DTI) parameters of lateral tricuspid annular motion, mean rate of pressure rise during RV contraction (mean dP/dt) and indexed right ventricular (RV) stroke volume (RVSVi) as RV function indices in repaired tetralogy of Fallot (TOF). Methods DTI evaluation was performed in 25 repaired-TOF patients, aged 11 ± 6, at rest and during dobutamine infusion and 20 controls at rest. Results TOF patients had lower (P < 0.05) peak systolic velocity (Sa) (11.4 ± 4 vs. 13.7 ± 3.1 cm/s), early diastolic velocity (Ea) (11 ± 3.1 vs. 16.3 ± 3.5 cm/s) and Sa/time from onset of QRS to Sa (Sa/Q-Sa) (68.8 ± 26.4 vs. 92.3 ± 29.4 cm/s 2 ) versus controls. Dobutamine increased (P < 0.01) Sa (11.4 ± 2.8-17.7 ± 4.7 cm/s), Ea (11 ± 3.1-15.6 ± 3.9 cm/s), late diastolic velocity (Aa) (8.4 ± 2-14.8 ± 5 cm/s), Sa/Q-Sa (68.8 ± 26.4-17 6.8 ± 84.5 cm/s 2 ), mean dP/dt (180 ± 74-537 ± 37 2 mmHg/s), and RVSVi (7.8 ± 3.9-11.9 ± 5.6 L/ min/m 2 ). RVSVi increase correlated (P < 0.01) with that in Sa (r = 0.6), Ea (r = 0.5), Sa/Q-Sa (r = 0.71), and mean dP/dt (r = 0.57) while mean dP/dt increase correlated strongly with Sa/Q-Sa increase (r = 0.88). Conclusion DTI evaluation of tricuspid annular motion during dobutamine infusion in repaired TOF correlates with dP/dt and RV stroke volume and may help in assessing RV function and reserve.

Three patients with anomalous origin of the left pulmonary artery (AOLPA) from the aorta underwent surgical correction. Two patients presented with an isolated malformation and one with an associated ventricular septal defect. One of the patients with an isolated malformation presented with an AOLPA from the descending thoracic aorta. Implantation of the AOLPA to the main PA trunk was performed by direct anatomosis in two patients and by interposition of a synthetic graft in the other patient with AOLPA from the descending aorta. All patients survived the operation. Low cardiac output was identified in one patient. The mean residual gradient through the anastomotic site was 21 mmHg at follow-up. We found almost 72 cases with AOLPA reported in the literature with a high mortality rate in patients not undergoing surgery. The most frequently found associated malformation is tetralogy of Fallot and ventricular septal defect. AOLPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients with AOLPA can undergo surgical repair with excellent results.

Objetivo -Relatar a experiência cirúrgica inicial de 4 casos com a utilização do auto-euxerto pulmonar para substituição da valva aórtica. Métodos -Quatro pacientes masculinos, brancos, com idades entre 23 e 46 anos, portadores de valvapatia aórtica, foram submetidos a substituição da valva aórtica pelo auto-enxerto pulmonar pela técnica de substituição total da raiz aórtica. Para a reconstrução da via de saída do ventrículo direito foram utilizados homo-euxertos pulmonares e aórtico conservados em solução de antibióticos. Todos tiveram controle pós-operatório com eco-doppler (ECO) e estudo hemodinâmica para a avaliação da função dos auto e homo-enxertos implantados. Conclusão -A cirurgia do auto-euxerto pulmonar deve ser implantada de forma definitiva em nosso meio. Aortic Valve Repicecement with Pulmonary Autograft (Ross Procedure). Methods -Four patients, all males, white, age between 23 and 46 years having aortic valve disease were submitted to aortic valve replacement with a pulmonary autograft using the root replacement technique. Right ventricular out flow tracts were reconstructed with antibiotic sterilized pulmonary or aortic homografts. All patients had control bidimensional eco-doppler (ECO) and hemodynamic study to evaluate the function of the implanted auto and homografts. Results -All patients had an excelent postoperative recovery, without the necessity of inotropic drugs. All presented in normal sinus rhythm. Postoperative ECO and hemodynamic studies revealed excelent function of the implanted autografts, without gradients in three and with a 15mmHg mean residual gradient in one case. There was no regurgitation in three cases and only trace aortic insufficiency in one. The right sided homografts also showed good function, with no gradient in two cases and mean systolic gradient of 6 and 8mmHg in the other two. Conclusion -The pulmonary autograft procedure should be implemented definitelly in our country.

Background: Patients with adult congenital heart disease (ACHD) have an increased risk of arrhythmic, sudden cardiac death. The subcutaneous implantable cardioverter defibrillator (S-ICD) provides a potentially safer alternative to transvenous ICDs in ACHD. Suitability for S-ICD depends on the surface ECG which is often abnormal in ACHD patients. This study investigates the proportion of ACHD patients who meet the screening criteria for S-ICD Methods A standard screening ECG was performed in 102 patients with complex ACHD (Tetralogy of Fallot, Fontan Circulation, Transposition of the Great Arteries). This process was repeated postexercise for patients who also had an exercise test. Results: Three quarters (75.4%) of ACHD patients meet screening criteria for an S-ICD with at least 1 suitable vector. The most common number of acceptable vectors in the eligible group was 2 (35% of total population). In only 12% were all three vectors suitable whilst 28% had only 1. The primary vector (equivalent of ECG lead III) was the most common suitable vector, found in 62% of participants who had appropriate sensing vectors. 25 (24.5%) patients failed to meet the S-ICD screening criteria. Of these, 14 had repaired tetralogy of Fallot. 92% of patients with a Fontan circulation met ECG screening criteria. Of those who had the protocol repeated following their cardiopulmonary exercise test (n=14), only 1 additional patient failed eligibility criteria. A quarter of ACHD patients do not meet eligibility criteria for the S-ICD. However, more than 90% of patients with a Fontan circulation are suitable for an S-ICD.

Objective: In fetuses with suspicion of congenital heart disease (CHD), assessment by segmental fetal echocardiography is of great importance. This study sought to examine the concordance of expert fetal echocardiography and postnatal MRI of the heart at a high-volume paediatric heart centre. Methods: The data of two hundred forty-two fetuses have been gathered under the condition of full pre- and postnatal and the presence of a pre- and postnatal diagnosis of CHD. The haemodynamically leading diagnosis was determined for each test person and was then sorted into diagnostic groups. The diagnoses and diagnostic groups were used for the comparison of diagnostic accuracy in fetal echocardiography. Results: All comparisons between the diagnostic methods for detection of congenital heart disease showed an “almost perfect” (Cohen’s Kappa &gt; 0.9) strength of agreement for the diagnostic groups. The diagnosis made by prenatal echocardiography showed a sensitivity of 90–100%, a specificity...

Diagnosis of congenital heart disease (CHD) in neonates with extra cardiac anomalies is effective in their clinical management and surgical outcomes. This study aimed to investigate the frequency of CHD in neonates with extra cardiac anomalies admitted to a neonatal intensive care unit (NICU). This retrospective, descriptive study included all neonates with extra cardiac anomalies admitted to the NICU of Ali Ibn Abitaleb Hospital in Rafsanjan, Iran, from March 2016 until the end of September 2018. Data were collected through a researcher-designed checklist including demographic and disease information for neonates and their parents. The checklist was filled out according to the neonates' medical records. Moreover, CHD was diagnosed using echocardiography. Finally, the data were analyzed using SPSS version 18. From the 58 neonates, 35 (60.35%) were boys and 23 (39.65%) were girls. The mean age of the neonates was 5.27±6.9 days. The prevalence of CHD in neonates with extra cardiac anomalies was 37.25% (n=19). The most prevalent CHD anomaly was PDA with a rate of 78.9%. The highest prevalence of CHD was associated with musculoskeletal (50%, n=6 from 12), and genitourinary (43.8%, n=7 from 16) abnormality, respectively. There was no statistically significant difference between the CHD and non-CHD neonates in terms of neonatal gender and parental characteristics. This study shows that 37.25% of neonates with extra cardiac anomalies have CHD anomalies; thus, it is recommended to refer all neonates with extra cardiac anomalies for cardiac examination.

A neonate with cyanosis at birth was found to have a rare type of tetralogy of Fallot. Echocardiography showed dextrocardia, left aortic arch with constricting and tortuous patent ductus arteriosus (PDA). Computed tomography angiography thorax revealed visceroatrial situs inversus, mirror image dextrocardia, tetralogy anatomy and tortuous right PDA arising from bovine brachiocephalic artery. In view of severe cyanosis, emergency division of PDA and modified right Blalock-Taussig shunt through median sternotomy were performed under cardiopulmonary bypass. Post-operatively, the sternum was left open and he developed pulmonary overcirculation requiring prolonged ICU stay. He was discharged well at Day 26 post surgery. This case highlights a rare association tetralogy of Fallot, dextrocardia and situs inversus, with concomitant unilateral right PDA and bovine aortic arch.

Adults with congenital heart disease represent an increasing population of patients that require repeated surgical interventions, most commonly for pulmonary valve replacement (PVR) and stenting [1]. Currently, the decision for treatment relies on symptoms, including arrhythmias and abnormal right ventricular volume and function [2], however the optimal timing for surgery remains unclear [3]. The aim of this work is to investigate the altered haemodynamic environment of the pulmonary bifurcation in adults with repaired tetralogy of Fallot, before and after pulmonary valve replacement. The overall objective is to derive a computational fluid dynamic metric to determine the optimal time for surgery. In this preliminary study, we present results in simplified geometries of the pulmonary trunk, focusing on the effects of geometry and flow conditions.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, for which patients require surgical intervention at a very young age. Although these patients have long survival rates, they are at risk of chronic complications and frequently require re-operations with the most common being pulmonary valve replacement (PVR). However, the decision for surgical intervention is currently based on clinical indications and the right timing for PVR remains ambiguous [1,2]. The overall objective of this work is to identify a computational metric that will help assess the right timing for surgical intervention in adults with repaired tetralogy of Fallot. This current study concerns a preliminary computational analysis of blood flow in simplified geometries of the pulmonary bifurcation. The focus lies on the effect of geometric and haemodynamic parameters on the wall shear stress patterns around the pulmonary bifurcation.

Understanding the haemodynamic environment of the pulmonary bifurcation is important in adults with repaired conotruncal congenital heart disease. In these patients, dysfunction of the pulmonary valve and narrowing of the branch pulmonary arteries are common and can have serious clinical consequences. The aim of this study was to numerically investigate the underlying blood flow characteristics in the pulmonary trunk under a range of simplified conditions. For that, an in-depth analysis was conducted in idealised two-dimensional geometries that facilitate parametric investigation of healthy and abnormal conditions. Subtle variations in morphology influenced the haemodynamic environment and wall shear stress distribution. The pressure in the left pulmonary artery was generally higher than that in the right and main arteries, but was markedly reduced in the presence of a local stenosis. Different downstream pressure conditions altered the branch flow ratio, from 50:50% to more realistic 60:40% ratios in the right and left pulmonary artery, respectively. Despite some simplifications, this study highlights some previously undocumented aspects of the flow in bifurcating geometries, by clarifying the role of the stagnation point location on wall shear stress and differential branch pressures. In addition, measurements of the mean pressure ratios in the pulmonary bifurcation are discussed in the context of a new haemodynamic index which could potentially contribute to the assessment of left pulmonary artery stenosis in tetralogy of Fallot patients. Further studies are required to confirm the results in patient-specific models with personalised physiological flow conditions.

Objective Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. Methods We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. Results We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). Conclusions Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment. ► Reduced cardiac function and elevated NT-proBNP, together with QRS duration shorter than 180 ms, might become parameters to consider in risk stratification in patients operated for TOF, especially considering the incidence of VA.

Background: Patients with tetralogy of Fallot (TOF) have a high survival rate 30 years after surgical repair, and generally enjoy a satisfactory quality of life. Many female patients experience pregnancy during adulthood, however the effects of pregnancy on the long-term cardiovascular outcome in this group of patients are not well known. We aimed to investigate the association of pregnancy and cardiac function with occurrence of ventricular arrhythmia (VA) in women operated for TOF. We included 80 patients recruited from the national database for patients diagnosed for TOF. All were examined with echocardiography, including strain echocardiography. We assessed mechanical dispersion of right ventricle (RV) as measure of heterogeneous contraction. Holter monitoring or implanted devices detected ventricular arrhythmias (VA), defined as non-sustained or sustained ventricular tachycardia or aborted cardiac arrest. Blood tests included N-terminal pro-brain natriuretic peptide (NT-proBNP). Results: In all, 55 (69%) women had experienced pregnancy (age 40 ± 9 years, parity median 1, range 1-4), while 25 (31%) women were nulliparous. The mean age was lower in nulliparous compared to those with children (30 ± 9 vs 40 ± 9, p < 0.01). VA was more prevalent in women who had experienced pregnancy (n = 16, 94%) compared to nulliparous (n = 1, 6%) (p = 0.02), and importantly also when adjusted for age [adjusted OR 9.8 (95% CI 1.2-79.1), p = 0.02]. RV mechanical dispersion was more pronounced in patients with VA [39.2 ± 14 ms vs. 49.6 ± 8 ms, p = 0.009, adjusted OR 2.1 (95% CI 1.3 -7.5), p = 0.01 adjusted for age]. Higher NT-proBNP was also a marker of VA [211 ng/L (127-836) vs. 139 ng/L (30-465), p = 0.007, adjusted OR 1.4 (95% CI 1.1 -1.8) p = 0.017 adjusted for age]. NT-proBNP >182 ng/L (normal values < 170 ng/L) optimally detected women with VA (p = 0.019), also independent of age [OR 7.2 (95% CI 1.7-30.1), p = 0.007]. History of pregnancy was associated with higher prevalence of VA among women with surgically corrected TOF. Right ventricular mechanical dispersion and NT-proBNP were age independent markers of VA. These findings may have importance for risk stratification and preconception counselling of these patients.