Aims: The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management. Methods and Results: Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug. Conclusions: Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Background— Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype. The most likely mechanism of inheritance was concluded to be autosomal dominant. We sought to identify the genetic pathogenesis in this family using a next generation sequencing approach. Methods and Results— Whole exome sequencing was performed in 2 cousins in this family using the Agilent SureSelect Human all Exon 51 Mb version 5 capture kit. Data were processed through an analytic in-house pipeline. Whole exome sequencing identified a missense mutation in FLNA (Filamin A), an actin-binding protein located at Xq28, mutations in which are associated with the skeletal phenotypes Frontometaphyseal dysplasia, Otopalatodigital, and Melnick–Needles syndrome, with X-linked periventricular nodular heterotopia and FG syndrome (Omim, 305450). Review of the phenotypes of those with the mutation in this family shows increased severity of the cardiac phenotype and associated skeletal features in affected males, consistent with X-linked inheritance. Conclusions— Although congenital heart disease is reported in families with mutations in FLNA , this is the first report of individuals being affected by Ebstein anomaly because of a mutation in this gene and details the concurrent skeletal phenotype observed in this family.

There is good evidence that morbidity and mortality increase for young persons (YP) following the move from paediatric to adult services. Studies show that effective transition between paediatric and adult care improves long-term outcomes. Many of the issues faced by young people across subspecialties with a long-term condition are generic. This article sets out some of the obstacles that have delayed the implementation of effective transition. It reports on a successful generic transition programme 'Ready Steady Go' that has been implemented within a large National Health Service teaching hospital in the UK, with secondary and tertiary paediatric services, where it is now established as part of routine care.

Congenital heart defects (CHDs) are the most common birth defect worldwide and are a leading cause of neonatal mortality. Nonsyndromic atrioventricular septal defects (AVSDs) are an important subtype of CHDs for which the genetic architecture is poorly understood. We performed exome sequencing in 13 parent-offspring trios and 112 unrelated individuals with nonsyndromic AVSDs and identified five rare missense variants (two of which arose de novo) in the highly conserved gene NR2F2, a very significant enrichment (p = 7.7 × 10(-7)) compared to 5,194 control subjects. We identified three additional CHD-affected families with other variants in NR2F2 including a de novo balanced chromosomal translocation, a de novo substitution disrupting a splice donor site, and a 3 bp duplication that cosegregated in a multiplex family. NR2F2 encodes a pleiotropic developmental transcription factor, and decreased dosage of NR2F2 in mice has been shown to result in abnormal development of atrioventricular septa. Via luciferase assays, we showed that all six coding sequence variants observed in individuals significantly alter the activity of NR2F2 on target promoters.

Major advances in paediatric cardiac surgery over recent years have resulted in an increased number of children with congenital heart disease (CHD) surviving into adulthood. Currently in Europe, the adult CHD population is greater than that of the paediatric CHD population. This changing prevalence and ageing congenital population brings with it multiple co-morbidities and new challenges in all areas of their medical, surgical and psychological management. This article gives an introduction to the common conditions presenting acutely to general physicians, long-term complications, treatment options and the long-term considerations for end-of-life care. Adult congenital heart disease patients are safest treated in a network with a multidisciplinary team approach.

S SA HEART CONGRESS 2013 98 TRACK 2: CATHETER INTERVENTIONS FROM FETUS TO ADULT Abstract no: 23 Device closures of large tubular patent ductus arteriosus with severe pulmonary hypertension: Analysis of 47 casesno: 23 Device closures of large tubular patent ductus arteriosus with severe pulmonary hypertension: Analysis of 47 cases Nurun Nahar and Fatema Begum Combined Military Hospital, Dhaka, Bangladesh Objective: Large patent ductus arteriosus can cause severe pulmonary hypertension (PHT) due to increased blood flow or increased pulmonary vascular resistance (PVR). Surgical ligations of these large tortuous hypertensive ducts are very difficult. Device closure is an alternative option and here we report 47 such cases which have undergone device closure in our centre. Patients and methods: A total of 47 patients had large tubular PDA with severe PHT from January 2009 December 2010. Retrospective review of data was done from records kept in the department. Results: The median age of the patient was 4.5 years (2 months 45 years) and median weight 9.7kg (3.5 60kg). Narrowest PDA diameter varied from 3.4 14mm with median of 4.8mm. Twenty nine patients were female, 18 were male. Pulmonary-to-systemic blood flow ranged from 1.1:1 6.7:1. Pulmonary vascular resistance ranged from 4.2 8.1 Wood units. Pulmonary arterial pressure ranged from 60/45 (50) 120/84 (97) mmHg. Systemic blood pressure ranged from 65/40 (48) 120/88 (99) mmHg. With 100% oxygen, pulmonary arterial pressure was reduced in 26 cases and remained the same in 9. In 12 cases device were implanted without this test. Minimum PDA device size was 5/4mm ADO 11 and maximum device size was 18mm Amplatzer septal occluder. Fourty three patients had complete occlusion and 4 had residual shunt. 3 cases had complete occlusion within 1st month. One patient developed haemolysis and managed with coil implantation. Embolisation was experienced in 1 case. On echocardiography, RV pressure reduced to normal by 3 months in all the cases except 2. Conclusion: Tubular PDA associated with severe PHT could be successfully closed by ductal occluders. After occlusion, pulmonary pressure had definite reduction and result in our centre is encouraging. Abstract no: 39 Impact of fenestration creation on managing patients with protein losing enteropathy complicating Fontan procedureno: 39 Impact of fenestration creation on managing patients with protein losing enteropathy complicating Fontan procedure Mashail Binobaidan and Jasim Abdulhameed Prince Sultan Cardiac Centre, Armed Force Hospital, Riyadh, Saudi Arabia Introduction: Protein losing enteropathy (PLE) is a well known complication following Fontan procedure and one way of managing it is to create fenestration if it is not present, or by enlarging if it is small to reduce the Fontan pressure and reduce venous congestion which results in intestinal protein loss. Aim: To evaluate the effect of such a procedure in our population. Method and material: From February 2006 October 2011, 9 patients who had fenestration creation due to the development of PLE were assessed with regard to clinical, laboratory result and haemodynamic effect preand post-procedure. Median age 7 years (4 21), median weight 23kg (15 52); male: female ratio 3.5:1 (male and 2 female); median saturation preand post procedure was 93% and 82% respectively; median albumin preand post-procedure was 18gm/ dl and 31gm/dl during the first 2 weeks and 36gm/dl thereafter; median pulmonary artery pressure before and after was 25mmHg (17 32mmHg) and 16mmHg. Results: (14 19mmHg) transpulmonary pressure gradient reduced from a median of 11mmHg 5mmHg; No immediate deaths; 2 patients needed re-dilatation; there were 2 (22%) late deaths; (1 patient had stent thrombosis 1 month after surgery followed by fulminant pulmonary embolism, though all patients were on anti-coagulant; the other 1 came to the emergency unit with sudden arrest but could not be resuscitated); 3 (33%) patients have persistent low albumin though the fenestration is patent. Conclusion: Transcatheter fenestration creation to manage PLE following the Fontan procedure is feasible and can be done in the cath lab with little morbidity and mortality and with beneficial effect; however late complications and completely solving PLE is of concern, especially if done late. Abstract no: 40 Right ventricular outflow tract stenting in the symptomatic infant with Tetralogy of Fallotno: 40 Right ventricular outflow tract stenting in the symptomatic infant with Tetralogy of Fallot Mashail Binobaidan and Jassim Abdulhameed Prince Sultan Cardiac Centre, Armed Force Hospital, Riyadh, Saudi Arabia Background: The debate continues regarding the initial management of cyanotic or duct-dependent infants with ToF especially those patients with pulmonary artery hypoplasia. While repair can and has been performed in these patients, it is associated with increased morbidity. Objective: We review the effectiveness of right ventricular outflow tract (RVOT) stenting in the symptomatic young infant with ToF. Methods and material: Clinical, echocardiographic, angiographic and haemodynamic data were reviewed for 13 patients who underwent 17 RVOT stenting procedures from March 2008 January 2012. There were 8 girls and 5 boys with median age 3 months and weight 3.5kg. The pulmonary valve was hypoplastic in all patients; median pulmonary valve diameter 3.1mm (range 2.7-5.2); and Z-score -5.5 (range -8.9 to -4.4). Results: RVOT stenting improved arterial oxygen saturation from a median of 60% (55 66%) 91% (82 94%). Median Z-score for the left pulmonary artery increased from -4.2 (-7.2 to -2.9) before stent implantation, to -1.8 (-4.6 to -0.8) at time of surgery. Median Z-score for the right pulmonary artery increased from -3.1 (-6.2 to -2.1) -0.5 (-2.1 to 0.2). There were no complications. Nine patients have undergone successful repair. There were no immediate or early deaths. Conclusions: In the symptomatic young infant with ToF, who abandoned surgery for any reason or at high risk, stenting of the RVOT provides a safe and effective management strategy, improving arterial oxygen saturation and encouraging pulmonary artery growth.

Background Incomplete formation of the partition between the two atrial chambers in the region of the oval fossa results in a range of defects, which extend from patent foramen ovale to large secundum atrial septal defects (ASDs). There is wide variation in the morphology of the latter lesions. The spatial orientation of the margins of ASDs relative to the persisting flap valve is not easily definable with standard echocardiographic imaging. Careful evaluation of the morphology is essential in optimizing successful transcatheter closure to minimize complications. The advent of three-dimensional transesophageal echocardiography has changed the understanding of the morphology of these defects and facilitated successful percutaneous closure. Methods Since 2007, over a 4-year period, transcatheter closure of ASDs was performed in 105 patients. Results During the study period, there were two instances of embolization of the device. The morphology of the defects in the patients with embolization was evaluated carefully, and an unusual spiral configuration of the flap valve relative to the rims of the oval fossa was noted. These findings were then found in four additional patients and serve as the focus of this report. To facilitate understanding of the unusual morphology, the clinical findings were compared with images showing the mechanism of development of the atrial septum in the mouse, revealing a striking similarity. Conclusions Although uncommon, spiral spatial orientation of the margins of ASDs predisposes to embolization of devices used for percutaneous closure. Standard cross-sectional techniques have limited use in identifying this variation. Understanding of the development of the atrial septum in the mouse heart may help explain the morphogenesis of the defect and the mechanism predisposing to embolization.

OBJECTIVES:To examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves.METHODS:We reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months).RESULTS:There were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively.CONCLUSIONS:PVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

OBJECTIVE:To review the use of three-dimensional echocardiography (3DE) with multiplanar reformatting (MPR) in children with congenital aortic stensosis undergoing percutaneous balloon aortic valvuloplasty to assess its accuracy in measuring the aortic valve annulus and any influence it may have on balloon sizing.METHODS:All percutaneous aortic balloon valvuloplasties performed from 01/01/2009 to 01/09/2011 were included in the study. All imaging performed for the procedure to determine the size of the aortic valve annulus and aid in balloon sizing was reviewed. The maximum diameter of the aortic valve annulus using two-dimensional echocardiography (2DE), 3DE with MPR, and angiography was recorded. The balloon size used in the procedure was recorded and the balloon to annulus ratio was calculated.RESULTS:A total of 27 procedures were included in the study. Age varied from 1 day to 156 months (mean age, 53 months) and weight from 2.8-58 kg (mean weight, 18.6 kg). Fourteen patients had 3DE with MPR available for analysis. The 3DE with MPR measurement (13.36 ± 5.4 mm) was not different from angiography (13.54 ± 6.4 mm; P=.803).The 2DE measurement was significantly different from angiography (11.72 ± 5 mm; P<.005). The balloon to annulus ratio based on angiographic measurements did not differ significantly between the patients with 3DE MPR and those without (0.94 ± 0.095 vs 0.91 ± 0.1; P=.468).CONCLUSION:3DE with MPR allows a more accurate assessment of the aortic valve annulus compared to 2DE, which may reduce the tendency to undersize balloon choice. 3DE with MPR did not significantly affect our balloon choice, which was largely based on angiographic measurements.

Our susceptibility to disease increases as we grow older. Robert Butler and colleagues argue that interventions to slow down ageing could therefore have much greater benefit than those targeted at individual disease

Background: We sought to determine the long-term performance of homograft and truncal valve after complete repair of common arterial trunk.Methods: From January 1964 to June 2008, 32 patients (median age, 14 days; range, 5 days to 2.5 years) underwent primary homograft repair of common arterial trunk. Twenty-four (75%) were neonates. The homograft used in the right ventricular outflow tract was aortic in 24 patients and pulmonary in 8 patients (mean diameter, 15.8 +/- 3.5 mm; median diameter, 16 mm [range, 8-24 mm]). The median follow-up was 24.5 years (range, 5.6 months to 43.5 years).Results: There were 3 hospital deaths and 1 late death. The actuarial survival at 30 years was 83.1% +/- 6.6%. Of the 28 survivors, 25 reoperations were performed in 19 (76%) patients. The mean and median times to homograft reoperation were 11.5 +/- 7.4 and 12.1 years (range, 1.0-26.1 years), respectively. Overall freedom from homograft reoperation after 10, 20, and 30 years was 68.4% +/- 8.7%, 37.4% +/- 9.5%, and 26.7% +/- 9.3%, respectively. Twelve patients retained the original homografts at a median follow-up of 16.4 years (range, 0-30.2 years). Six underwent a truncal valve replacement with a mechanical prosthesis at a median of 10.5 years (range, 3.4-22 years) after truncus repair. Freedom from truncal valve replacement at 10 and 30 years was 93.1% +/- 4.7% and 81.8% +/- 8.9%, respectively. In the 22 surviving patients who did not undergo truncal valve replacement, the peak truncal valve gradient was 8.9 +/- 8.3 mm Hg at a median follow-up of 24.5 years (range, 5.6 months to 32.9 years). At the last follow-up, 27 (96.4%) patients had good left ventricular function, and 24 patients (85.7%) were New York Heart Association class I.Conclusions: Oversizing the homograft at the time of the initial repair can lead to a homograft lasting more than 12 years. During long-term follow-up, 20% of patients require truncal valve replacement. (J Thorac Cardiovasc Surg 2010;140:325-9)

Background. The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair.Methods. Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle.Results. There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients.Conclusions. Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24±5.8years, (range 16.7–39.8) and mean Fontan duration was 16.8±4.8years (range 7.3–28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1–22) arterialised nodules, mean size 1.8cm (range 0.5 to 3.2cm). All nodules were located in the liver periphery, their outer aspect lying within 2cm of the liver margin. Patients with nodules had higher mean RA pressures (18mmHg±5.6 vs. 13mmHg±4, p=0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70%±11 vs. 67%±9 and 92%±10 vs. 94%±4, p>0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.

An abstract is not available for this content so a preview has been provided. Please use the Get access link above for information on how to access this content.

BACKGROUND:Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS:A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS:Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS:Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.

BACKGROUND:The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. OBJECTIVE:To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. METHODS:Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years. RESULTS:Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). CONCLUSIONS:Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

AIMS:Patients with cryptogenic embolic events and a patent foramen ovale (PFO) are at risk of paradoxical embolism causing recurrent cerebral events; however, transcatheter PFO closure remains controversial. The aim of this multicentre trial was to demonstrate the feasibility and safety of transcatheter closure of PFO with the HELEX Septal Occluder.METHODS AND RESULTS:The study enrolled 128 patients (66 female; mean age, 50 years). Mean (+/-SD) PFO size was 10+/-3.7 mm; 38 patients also had an atrial septal aneurysm. Device implantation was successful in 127 patients. Device-related events during implantation or follow-up were device embolisation, wire-frame fracture, and retrieval cord breaks (two cases each; no sequelae). Other adverse events included atrial arrhythmia (two patients), migraine, convulsion, and transient ischaemic attack (one case each). There were no recurrent strokes, deaths, perforations, or accumulations of thrombi on the device. Within a mean follow-up period of 21+/-11 months, complete PFO closure using one device was achieved in 114 patients (90%). Five patients with a moderate to large residual shunt received a second device.CONCLUSION:The HELEX Occluder can be used for PFO closure. Device- and procedure-related complications are rare. The closure procedure appears to reduce recurrence rates of stroke and transient ischaemic attack.

OBJECTIVE:To define the anatomic characteristics of the congenitally malformed and severely stenotic aortic valve using trans-thoracic real time three-dimensional echocardiography, and to compare and contrast this with the valvar morphology as seen at surgery.DESIGN:Prospective cross-sectional observational study.SETTING:Tertiary centre for paediatric cardiology.METHODS:All patients requiring aortic valvotomy between December 2003 and July 2004 were evaluated prior to surgery with three-dimensional echocardiography. Full volume loop images were acquired using the Phillips Sonos 7500 system. A single observer analysed the images using "Q lab 4.1" software. The details were then compared with operative findings.RESULTS:We identified 8 consecutive patients, with a median age of 16 weeks, ranging from 1 day to 11 years, with median weight of 7.22 kilograms, ranging from 2.78 to 22 kilograms. The measured diameter of the valvar orifice, and the number of leaflets identified, corresponded closely with surgical assessment. The sites of fusion of the leaflets were correctly identified by the echocardiographic imaging in all cases. Fusion between the right and non-coronary leaflets was identified in half the patients. Dysplasia was observed in 3 patients, with 1 patient having nodules and 2 shown to have excrescences. At surgery, nodules were excised, and excrescences were trimmed. The dysplastic changes correlated well with operative findings, though statistically not significant.CONCLUSION:We recommend trans-thoracic real time three-dimensional echocardiography for the assessment of the congenitally malformed aortic valve, particularly to identify sites of fusion between leaflets and to measure the orificial diameter. The definition of nodularity, and the prognosis of nodules based on the mode of intervention, will need a comparative study of patients submitted to balloon dilation as well as those undergoing surgical valvotomy.

The objective of this study was to evaluate the effectiveness and safety of esmolol-induced negative ino- and chronotropism during stent/balloon angioplasty for aortic coarctation. Balloon angioplasty and stent placement have become widely accepted therapies for native and recurrent coarctation of the aorta (CoA). Trauma to the vessel wall and stent migration related to forward displacement of the balloon and/or stent by cardiac output, are the most common complications. Controlling stroke volume and heart rate may assist in balloon stability and accurate deployment of stents. All methods currently used to achieve this have significant limitations. We describe our experience using esmolol to control stroke volume and heart rate during balloon/stent angioplasty of CoA. We performed a retrospective review of all patients who had intravenous esmolol during percutaneous treatment of CoA. Six interventions were performed in six patients: coarctation stent angioplasty in five patients (two native coarctation) and balloon angioplasty alone in one patient. The median systolic blood pressure achieved during the procedure was 65 mmHg (range, 57–75) representing a median reduction of 40 mmHg (range, 20–80; p = 0.008) from baseline. The median heart achieved was 50 beats/min (range, 20–80), representing a median reduction of 20 beats/min (range, 15–90, p = 0.048) from baseline. Optimal stent position was obtained in all patients. Intravenous esmolol controls periprocedural hemodynamics effectively and safely during percutaneous therapy for aortic coarctation, thereby aiding accurate stent placement. Further evaluation of its use during other percutaneous left heart interventions is required.