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Graphical abstract, Highlights • PVL after TMVR with a Tendyne device can result in severe intravascular hemolysis. • SAM of the mitral valve can cause anterior PVL following Tendyne TMVR. • TAVR can relieve LVOT obstruction caused by SAM after Tendyne TMVR. • Pre-, peri-, and postprocedural imaging is key in decision making.

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

Background Scimitar syndrome is a rare combination of cardiopulmonary abnormalities found in 1–3 per 1000 live births. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is only found in 1 in 250–400 congenital heart disease patients. Objective We aimed to investigate the incidence of left circumflex ALCAPA within our referral center’s cohort of scimitar syndrome patients. Materials and methods A review of medical records, cardiac imaging and operative notes from all patients diagnosed with scimitar syndrome at our center between 1992 and 2016 was undertaken and all imaging reviewed. Results Fifty-four patients with scimitar syndrome and imaging were identified. Of these, 3 patients (1 male and 2 female) with ALCAPA were identified, representing an incidence of 5.5% (95% confidence interval [CI] 0–11.67%). In all three cases, the anomalous coronary arising from the pulmonary artery was the left circumflex coronary artery (LCx) and the point of origin was close to the pulmonary arterial bifurcation. Conclusion We hypothesize that the prevalence of LCx-ALCAPA, in the setting of scimitar syndrome, may be greater than previously thought. We suggest that any patient with scimitar syndrome, especially with evidence of ischaemia, should be investigated for ALCAPA. Given its noninvasive nature and simultaneous imaging of the lungs, we suggest that cardiovascular CT is the most appropriate first-line investigation for these patients.

Phosphodiesterase 3 inhibitors have been used successfully in pediatric patients with acute or chronic myocardial dysfunction over the last two decades. Their protracted continuous intravenous administration is associated with risk of infectious and thromboembolic complications. Weaning intravenous medication and starting oral angiotensin-converting enzyme (ACE) inhibitors and/or beta-blockers can be challenging. We reviewed retrospectively hospital records of 48 patients receiving oral enoximone treatment in a single tertiary pediatric cardiac center between November 2005 and April 2014. Failure to wean from intravenous milrinone infusion and/or intolerance of ACE inhibitors and/or beta-blockers was indications for oral enoximone treatment. Age of the patients ranged between 0.5 and 191 months (median 7.5 months) at the time of starting enoximone treatment. There were 14 patients (29 %) with left ventricular dysfunction due to myocarditis or dilated cardiomyopathy and 34 patients (71 %) with myocardial dysfunction complicating congenital heart disease. Fifteen (44 %) of these 34 patients had left ventricular dysfunction, 13 (38 %) right ventricular dysfunction, and in 6 (18 %) both ventricles were failing. Duration of oral enoximone treatment was between 3 days and 34 months (median of 2.3 months). Myocardial functional recovery allowed for weaning of enoximone treatment in 15 patients (31 %) after 6 days-15 months (median 5 months). No adverse hemodynamic effects were noted. Blood stained gastric aspirates encountered in two patients resolved with concomitant milk administration. Based on our limited experience, oral enoximone is a well-tolerated and promising alternative to intravenous medication and/or other commonly used oral medications in selected pediatric patients with chronic heart failure.

Total anomalous pulmonary venous connection (TAPVC) to unroofed coronary sinus is a rare cardiac condition. We report the first case of antenatal diagnosis which had a rapid and fatal neonatal course due to spinal muscular atrophy (SMA) type I (Werdnig-Hoffmann disease). The diagnosis of TAPVC with unroofing of the coronary sinus was made at 26 weeks’ gestation, although a dilated inferior caval vein had been recognised at 23 weeks’ gestation. Due to profound hypotonia after birth, genetic review followed by muscle biopsy was performed due to presumptive diagnosis of SMA. The infant deteriorated rapidly, became ventilator dependent and died at the age of 6 weeks. The worst types of SMA are associated with only one copy of SMN2 (survival motor neuron 2) protein, which was also the case of our infant. Although a powerful association with congenital heart defects was described, it has not been reported with TAPVC to unroofed coronary sinus. Moreover, this cardiac condition has not been previously recognised antenatally.

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

Objective: To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood. Design and setting: Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and Sweden. Patients: Cases of PVS presenting between 1 January 1995 and 31 December 2004 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed. Results: 58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 0.99, p = 0.023). Conclusions: PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.

A wealth of experience has been gained in the management of atrioventricular septal defect (AVSD) since the first complete correction of this malformation in 1955. The success of surgical therapy followed an enhanced understanding of morphology and physiology as well as major improvements in imaging of this congenital heart defect. Therapeutic success in the management of patients with AVSD has been extended to include those with associated lesions such as tetralogy of Fallot, double outlet right ventricle and relative degrees of ventricular hypoplasia. Although operative mortality is low and long-term survival is relatively good, important detrimental residual or AVSD-related complications such as left atrioventricular valve regurgitation, left ventricular outflow tract obstruction still carry significant late morbidity in a proportion of patients. This article reviews our current understanding of the morphology of this defect, aspects of diagnosis and surgical treatment options.

Fascination with pulmonary atresia and intact ventricular septum (PAIVS) reflects its diversity and evolving management strategies. We will discuss PAIVS with situs solitus and concordant atrioventricular and ventriculoarterial connections. Usually the cavity of the right ventricle (RV) is small (hypoplastic), the RV myocardium is thickened, and the RV pressure is at supra systemic levels. In a minority with severe tricuspid regurgitation the RV is dilated and thin walled and postnatally the RV pressure is low. Also contributing to the morphological heterogeneity are the size and structure of the tricuspid valve (TV), whether RV outflow tract obstruction (RVOTO) is muscular or valvar, and the presence or absence of coronary artery abnormalities. Postnatal survival initially is dependent on persistence of the arterial duct. Management strategies are influenced by the nature of the RVOTO and whether the RV is considered suitable for eventual inclusion in the circulation. Should there be a biventricular, univentricular or one-and-a-half ventricle repair? In a 20 year population based study in Sweden (1980–1999)1 84 children were born with PAIVS, giving an incidence of 4.2 per 100 000 live births. In the UK/Eire from January 1991 to December 1995 all fetuses and infants born with PAIVS were documented in a multicentre, population based collaborative study.2 There were 183 live births with an overall incidence of 4.5 per 100 000 live births. Significant (p = 0.01) regional differences were found with an incidence of 4.1 per 100 000 in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 per 100 000 in Northern Ireland. There were 86 fetal diagnoses made at a mean of 22 weeks gestation leading to elective termination of pregnancy (TOP) in 61%, four intrauterine deaths (5%), and 29 (34%) live births. If there were no TOP or spontaneous deaths, the incidence at birth would be 5.6 per 100 000 in …

There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

Objective: To determine the safety and effectiveness of cutting balloon angioplasty for pulmonary vein stenosis (PVS). Design and setting: Retrospective review of case notes and cardiac catheterisation data at the Royal Brompton Hospital. Main outcome measures: Diameter of pulmonary vein, tricuspid regurgitant jet velocity on echocardiogram, and percutaneous oxygen saturation before and after cutting balloon angioplasty. Results: Three patients had congenital PVS and three had PVS associated with total anomalous pulmonary venous drainage. A total of 27 PVSs were treated during 12 catheterisation procedures. Median patient age at the time of procedure was 12.5 months (range 1.5–36 months) and weight was 7.1 kg (range 2.8–11.1 kg). Minimum pulmonary vein diameter increased significantly on angiography after cutting balloon angioplasty, from mean (SD) 2.3 (0.7) mm to 4.2 (1.9) mm, mean of differences 1.9 mm (95% confidence interval (CI) 0.9 to 2.9 mm, p = 0.0013). Mean (SD) oxygen saturation rose from 79.6 (12.9)% to 83.9 (9.0)%, mean of differences 4.3% (95% CI 0.7% to 8.0%, p = 0.0238). All children’s symptoms improved subjectively. Tricuspid regurgitant jet velocity did not change significantly. The longest time interval before repeat intervention was six months. There were no acute deaths; one patient had a small pulmonary haemorrhage and developed a small aneurysm adjacent to the site of angioplasty. Conclusion: Cutting balloon angioplasty is safe in the palliation of PVS in children. It gives some acute relief but often needs to be repeated, as improvement is rarely sustained.

The first ever transcatheter valve implantation was performed by Philip Bonhoeffer in 2000 when he implanted the first version of the Melody valve into a failing prosthetic conduit from the RV to the pulmonary artery.1 Since then transcatheter pulmonary valve implantation (TCPVI) has become an attractive and nowadays widely used alternative to surgical pulmonary valve replacement.2 While this procedure was initially designed for treatment of pulmonary conduit failure, its ‘off label’ applications have recently become more common. Smaller case series have been published reporting on strategies and techniques to implant a transcatheter valve into the ‘native’, compliant outflow tract or into failing tricuspid valve prostheses.3 ,4 The potential advantages of TCPVI over open-heart surgery are obvious (eg, shorter recovery time from the procedure and reduction of the number of cardiopulmonary bypass procedures per lifetime), but concerns remain about the longevity of the valve and above all about the risk of infective endocarditis (IE). van Dijck et al 5 report the incidence of IE after TCPVI using the Melody valve system in their institution. This on its own is an important addition to the existing literature, but the main value of this publication relates to the fact that these data are reported in comparison with surgical implantation of homograft and Contegra conduits. …

The anatomy of atrial and ventricular septal defects is reviewed with reference to the normal cardiac septum. Owing to the limited extent of the atrial septum, true septal defects are confined to the area of the oval fossa. The sinus venosus and coronary sinus defects are interatrial communications being outside the confines of the atrial septum and close to important atrial structures. The description of ventricular septal defects as perimembranous, muscular and doubly committed, and juxtaarte rial defects highlights the anatomy of the margins, the location, and proximity to the conduction system and valvar structures. Malalignment of septal structures can complicate the anatomy of ventricular septal defects producing inlet or outlet obstruction.

To assess right ventricular function in adults and children with Ebstein's anomaly.Prospective study.Tertiary referral centre.Fifteen patients (8 adults and 7 children) with Ebstein's anomaly and 14 healthy controls.Transthoracic echocardiography was performed in all. Right ventricular function was assessed from long axis M-mode recordings of the right atrio-ventricular free wall. Total systolic excursion as well as peak shortening and lenghtening rates of the right ventricle were measured.Children and adult patients with Ebstein's anomaly differed in terms of age at diagnosis, the adult group having been diagnosed later 19.8+/-15.8 vs. 5.9+/-6.2 years, P0.05. Measures of right ventricular long axis function in children with Ebstein's anomaly showed a significantly reduced systolic excursion 1.4+/-0.4 vs. 2.4+/-0.53 cm, P0.05 and peak lenghtening rate; early 8.04+/-4.3 vs. 11.8+/-2.4 cm/s and late 6.14+/-3.6 vs. 10.6+/-4.3 cm/s, P=0.05 compared to controls. In contrast, measurements of right ventricular long axis function in adults with Ebstein's anomaly showed no significant difference when compared to the control group. Transtricuspid Doppler flow velocities were not different between patient's groups and corresponding controls.The right ventricle assessed by this simple, non-invasive technique reveals a significantly reduced systolic and diastolic function in children with Ebstein's malformation compared to controls but a 'normal' right ventricular function comparable to controls in adult patients. Significant right ventricular dysfunction in children with Ebstein's anomaly could account for their earlier presentation. Long term follow up of the right ventricular abnormalities is needed in such children to discover more about the natural history of the disease.

OBJECTIVE—To evaluate the feasibility of anatomical correction based on morphological and echocardiographic findings in patients and preserved hearts with discordant atrioventricular connections. DESIGN—A retrospective study with clinicomorphological correlations to assess potential contraindications for anatomical correction in the setting of discordant atrioventricular connections. SETTING—A tertiary referral centre for congenital heart disease. MATERIAL—25 specimens and 53 patients unified by presence of discordant atrioventricular connections. METHODS—The potential contraindications for anatomical correction were first evaluated on the basis of morphological findings in all 25 specimens with discordant atrioventricular connections collected in the department museum, including study of the major coronary arterial patterns in 20. These contraindications were then sought in a population of 53 patients examined echocardiographically between January 1992 and October 1997. RESULTS—At least one lesion was discovered that might have contraindicated anatomical correction in 14 of the specimens and in 16 of the patients. The most common lesions that might militate against the anatomical approach were severe Ebstein's malformation or straddling and overriding of the tricuspid valve, each when combined with hypoplasia of the morphologically right ventricle. Other potential contraindications were atrioventricular septal defect with common atrioventricular junction, and obstruction of the left ventricular outlet combined with a restrictive ventricular septal defect, although these may be overcome with increasing experience and expertise. CONCLUSIONS—According to the morphological and echocardiographic findings, at least 10 hearts and 37 patients would have produced no anatomical problems for the type of surgical correction in which the morphologically left ventricle is restored its rightful role as the systemic pumping chamber. Keywords: atrioventricular discordant connections; congenitally corrected transposition; double switch operation

Objective—To study patients with atrioventricular septal defect to determine the pathognomonic morphological features of the lesion and the relation between the septal structures and the atrioventricular junction. Setting —Tertiary level paediatric cardiology centre. Methods—Cross sectional echocardiograms from 60 patients were reviewed using qualitative and quantitative analysis. The unifying feature was the presence of a common atrioventricular junction. The overall dimensions of the septal defect were determined and related to the plane of the common junction; the extent of both the atrial and the ventricular septal components was then measured according to the site of closure of the bridging leaflets. Results—In 48 cases, the common junction was guarded by a common valvar orifice, but in 12 cases there were separate right and left valvar orifices. Irrespective of the valvar morphology, no significant difference was found between the groups in terms of the dimensions of the atrial and ventricular septal components. In all patients, the hole permitting shunting at atrial level extended below the plane of the atrioventricular junction, with a variable position of the leading edge of the atrial septum itself. Conclusions—The atrioventricular junction is a common structure irrespective of valvar morphology. In spite of the presence of unequivocal shunting at atrial level, the atrial septum is usually a well formed structure, even extending in some below the level of the common atrioventricular junction. Keywords: atrioventricular canal malformation; endocardial cushion defects; level of shunting; morphology

We present the case of a neonate with pulmonary atresia and persistent bilateral patent ductus arteriosus imaged by gated multidetector CT. Traditionally, these patients have been assessed preoperatively with invasive angiocardiography or with cardiovascular magnetic resonance under sedation. Our case illustrates that contemporary cardiovascular CT techniques can now be used for preoperative evaluation with minimal radiation penalty, obviating the risks of sedation or cardiac catheterization.

Aims We analysed the type and mechanism of supraventricular arrhythmias encountered in a series of symptomatic adults with atrial isomerism undergoing catheter ablation procedures. Methods and results The study population included consecutive adults with atrial isomerism who had previously undergone surgical repair or palliation of the associated anomalies. Patients underwent electrophysiological study for symptomatic arrhythmia in our institution between 2010 and 2012 using magnetic navigation in conjunction with CARTO RMT and three-dimensional (3D) image integration. Eight patients (five females) with a median age of 33 years [interquartile range (IQR) 24–39] were studied. Access to the cardiac chambers of interest was obtained retrogradely via the aorta using remotely navigated magnetic catheters in six patients. Radiofrequency ablation successfully targeted twin atrioventricular (AV) nodal reentrant tachycardia in two patients, atrial fibrillation (AF) in three, focal atrial tachycardia (AT) mainly originating in the left-sided atrium in four patients, and macro-reentrant AT dependent on a right-sided inferior isthmus in three patients. The median fluoroscopy time was 3.0 min (IQR 2–11). After a median follow-up of 10 months (IQR 6–21), five of the ablated patients are free from arrhythmia; two patients experienced episodes of self-terminated AF and AT, respectively, within one month post-ablation; the remaining patient had only non-sustained AT during the electrophysiological study and was managed medically. Conclusion Various supraventricular tachycardia mechanisms are possible in adults with heterotaxy syndrome, all potentially amenable to radiofrequency ablation. The use of remote magnetic navigation along with 3D mapping facilitated the procedures and resulted in a short radiation time.

OBJECTIVE--To show that abnormal systemic venous channels in patients who undergo cavopulmonary anastomoses can become manifest and haemodynamically important only after surgery despite detailed preoperative investigation. DESIGN--Descriptive study of patients fulfilling the above criteria selected from hospital records over the past three years. SETTING--A tertiary referral centre. PATIENTS--Of the three cases identified, two were isomeric, one with left atrial isomerism and hemiazygos continuation of the inferior vena cava who underwent bilateral bidirectional Glenn anastomoses and one with right isomerism who underwent total cavopulmonary anastomosis. Case 3 had absent left atrioventricular connection with a hypoplastic left lung and underwent a classic right Glenn procedure. All three cases presented with progressive cyanosis in the early postoperative period. INTERVENTIONS AND RESULTS--Postoperative angiography in case 1 showed a remnant of a left inferior vena cava draining to the atrium to have become grossly dilated causing cyanosis, which resolved after redirection of this vessel and of the hepatic veins into the right pulmonary artery with an intra-atrial baffle. Cyanosis in case 2 was caused by intra-hepatic shunting to a hepatic vein draining to the left of the intra-atrial baffle. The diagnosis was made at necropsy, being overlooked on postoperative angiography. Repeat angiography in case 3 showed progressive dilatation of a small left superior vena cava to coronary sinus. Test occlusion with a view to embolisation revealed hitherto an undemonstrated hemiazygos continuation of inferior caval to brachiocephalic vein. The patient underwent surgical ligation of these two venous channels. CONCLUSIONS--Despite appropriate investigation some "abnormal" venous pathways manifest themselves, dilate, and become haemodynamically important only after surgical cavopulmonary anastomoses. In the presence of early postoperative cyanosis "new" systemic venous collateral channels should be considered as a possible cause, which may require reintervention.

Anomalous origin of the left coronary artery from the pulmonary trunk usually presents in early infancy with congestive cardiac failure due to ischemic myocardial dysfunction. The diagnosis can usually be made by parasternal short-axis sections of the great arteries. The most characteristic finding is reversed flow in the left coronary artery demonstrated by color flow Doppler. Usually, the anomalous coronary artery connects to the pulmonary trunk at one of the sinuses, but in some cases the connection may be to the more distal pulmonary arteries. A less common presentation of this condition is in late childhood or early adult life, usually with left ventricular dysfunction and mitral regurgitation. There is almost always reversed blood flow in the left coronary artery, representing a left-to-right shunt from the right coronary artery into the pulmonary trunk.

OBJECTIVE--To obtain angiographic views in tetralogy of Fallot that can show whether or not an anomalous coronary artery passes anterior to the right ventricular outflow tract. DESIGN--(a) A 10 year retrospective review of all patients who underwent repair of tetralogy of Fallot up to December 1990; (b) a prospective study of 30 children undergoing routine cardiac catheterisation. PATIENTS AND METHODS--295 cases in whom standard angiographic views had been used were reviewed retrospectively. Thirty non-consecutive children with tetralogy of Fallot were studied prospectively, including one child previously studied in whom diagnosis of an unsuspected anomalous coronary artery was made only at operation. The aortogram was performed with > or = 45 degrees caudocranial and 20 degrees-30 degrees left anterior oblique angles. SETTING--Tertiary referral centre. RESULTS--Ten of the 295 cases reviewed were shown to have a coronary vessel traversing the right ventricular outflow tract. In one case the diagnosis was suspected before operation but it was missed in the others. Even in retrospect we could not be certain of the precise anatomy with the use of standard angiographic views. In the prospective study the caudocranial aortogram showed the aortic valve face on in all the patients. The right ventricular outflow tract lay in a left and anterior (seen as superior) position in relation to the aortic root. Thus any vessel crossing the outflow tract could be identified. Identification of the aortic cusps allowed precise definition of the origin of the coronary arteries. All but four had normal origin and course of the coronary arteries. Four had paired left anterior descending arteries (including the restudied patient), in all cases with a large vessel originating from the right coronary artery passing across the right ventricular outflow tract. CONCLUSIONS--Important anomalies of the coronary arteries in tetralogy of Fallot may remain undiagnosed if standard angiographic projections are used. Aortography with > or = 45 degrees caudocranial and 20 degrees-30 degrees left anterior oblique angles allows precise definition of the anatomy and certainty as to whether any major vessel crosses the right ventricular outflow tract. Interpretation, however, can only be correct if the projection is technically adequate with a view of the aortic valve face on. Furthermore, a normal bifurcation of the left main stem does not exclude a second left anterior descending artery crossing the pulmonary outflow tract.

SummaryFormation of aortic aneurysms is an important and not infrequent sequel of surgical repair of aortic coarctation. Detection is often haphazard and fatal ruptures are reported. We report a series of cases where resonance imaging identified aneurysmal dilatation of the thoracic aorta after either patch aortoplasry or subclavian flap repair of aortic coarctation. In one, rupture of the aneurysm occurred five months following the initial resonance study. Repeat imaging confirmed the clinical suspicion of rupture and successful surgical resection was subsequently performed. Magnetic resonance is a practical and reproducible imaging technique which is particularly valuable in the identification of abnormalities within the thoracic aorta following surgical aortoplasty or interventional procedures for aortic coarctation. As a method of routine surveillance in these groups of patients, it is superior to any alternative technique.

A 66-year-old man was scheduled for percutaneous balloon valvuloplasty of pulmonary valvular stenosis under general anesthesia in the cardiac catheterization laboratory. The patient had a significant history of cardiorespiratory disease. In 1944, his family physician detected a heart murmur, and in 1955, the patient was referred to a cardiologist and told he had “a narrowed valve.” The patient was then lost to follow-up, and his old hospital records were lost. The patient claimed to have been asymptomatic until he sustained an inferior myocardial infarction in 1999 and was referred to this hospital for further investigations. On admission, the patient admitted to smoking 15 cigarettes per day until his myocardial infarction, and his exercise tolerance was reduced to less than 500 meters during the last few years. His medications were aspirin and atenolol. On examination, the patient was noted to be cyanotic, had clubbing of the fingers, had a raised jugular venous pressure, and had bilateral basal crepitations on chest auscultation. The patient had a soft ejection systolic murmur with a normal second heart sound, and there was no right ventricular heave. Further investigations revealed polycythemia (hemoglobin, 19 g/dL; hematocrit, 57.3%), mild renal impairment (serum creatinine, 143 mmol/ L), and arterial blood gases and lung function testing as shown in Tables 1 and 2. A chest radiograph was normal, and a 12-lead electrocardiogram showed T-wave inversion in leads I through III and no evidence of right ventricular hypertrophy. At cardiac catheterization, the patient was found to have moderate left ventricular dysfunction, with a mild stenosis of the left anterior descending coronary artery and complete occlusion of the dominant right coronary artery. There was a severe pulmonary valve stenosis with a peak-to-peak systolic gradient of 90 mmHg (Fig 1). A small atrioseptal defect or patent foramen ovale was noted and confirmed with transesophageal echocardiography. Transesophageal echocardiography did not show any evidence of right or left ventricular dysfunction, and left ventricular ejection fraction was calculated to be 60%. Oxygen saturations and pressure measurements are shown in Tables 3 and 4. It was decided that the pulmonary stenosis should be treated by percutaneous balloon valvuloplasty. Standard anesthetic monitoring was applied, including a 5‐lead electrocardiogram and direct arterial blood pressure measurement via a 20G radial artery catheter inserted under local anesthesia. Pulse oximetry in air showed a saturation (SpO2) of 92%. After preoxygenation (SpO2 increased to 100%), anesthetic induction consisted of fentanyl, 100 g; etomidate, 16 mg; and vecuronium, 8 mg. After endotracheal intubation, the lungs were ventilated with oxygen, air, and isoflurane, 0.8% to 1.4%, for maintenance of anesthesia. The right internal jugular vein was cannulated with 2 long 14G catheters for monitoring of central venous pressure and easy administration of drugs. During the procedure, 1000 mL of lactated Ringer’s solution was infused. Anesthesia was uneventful, and the angiographic findings compared well with the previous measurements. Balloon pulmonary valvotomy using a 25 mm 40 mm balloon resulted in the peak-to-peak gradient falling to 12 mmHg (Figs 2 and 3, Table 5). The patient’s other cardiovascular parameters were unchanged, and PaO2 improved immediately to 9.65 kPa. The procedure time was 15 minutes. The small atrial septal defect was of little or no significance, and no further procedures were carried out. Neuromuscular blockade was reversed with glycopyrrolate, 500 g, and neostigmine, 2.5 mg, and the patient’s lungs were ventilated with oxygen until adequate spontaneous ventilation was restored and the patient’s trachea could be extubated. Total anesthetic time was 45 minutes. The patient was transferred to the postanesthesia care unit to await transfer to the ward. Within 5 minutes of admission to the postanesthesia care unit, the patient developed a tachycardia (120 beats/min), his respiratory rate increased to 30 breaths/min, and SpO2 decreased to 85%, despite supplemental oxygen by facemask.

SummaryWe describe a new method of transvenous laser or radiofrequency perforation of the pulmonary valve with subsequent balloon pulmonary valvoplasty. The technique obviates the need for arterial catheterisation and considerably shortens the time needed for the procedure. Uncomplicated and successful relief of pulmonary valvar atresia was achieved in both patients attempted, although one ultimately required a Blalock-Taussig shunt because of increasing muscular obstruction of the right ventricular outflow tract.

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

SummaryUsing combined epicardial echocardiography and high-fidelity intraventricular pressure recordings, the acute effects of transition to a Fontan circulation were studied in 10 patients. Measurements were made before and after cardiopulmonary bypass. The Fontan operation had no significant effect on load-dependent indices, or on a load independent index (Vmax) of ventricular systolic function. Large changes were observed in ventricular geometry. Maximum and minimum cavity dimensions were reduced, while maximum and minimum thicknesses of the posterior wall were increased.While simultaneous Doppler/intraventricular pressure measurements suggested that the compliance of the ventricle was unchanged, the pattern of atrioventricular flow changed from predominantly early diastolic (E wave) to predominantly atrial systolic (A wave) as a result of surgery in four patients. The time constant of ventricular relaxation was prolonged in all patients and five developed Doppler echocardiographic evidence of incoordinate relaxation of the ventricle, with intraventricular flow occurring during isovolumic relaxation. Thus, the Fontan operation causes acute hypertrophy of the systemic ventricle due to a sudden reduction in ventricular preload in the presence of a maintained shortening fraction. While global ventricular diastolic compliance may be unchanged by the operative procedure, filling of the ventricle during early diastole is altered as a result of incoordinate relaxation and a prolonged time constant of ventricular relaxation.

A 9-month-old infant with a clinical diagnosis of double outlet from the right ventricle, subaortic ventricular septal defect, and muscular subpulmonary obstruction underwent surgical repair. The defect was corrected and the right ventricular infundibulum enlarged with a patch. Eight hours after the operation there was a sudden collapse which rapidly progressed to cardiac arrest and death. Postmortem examination of the heart confirmed the clinical diagnosis but noted that the subpulmonary obstruction was caused by a "windsock" deformity of the tricuspid valve which was not identified at operation. The variants of malformations of the tricuspid valve causing subpulmonary obstruction are reviewed.

Introduction A male infant was diagnosed and treated for Group B Streptococcus meningitis from day 10 of life after uncomplicated pregnancy and delivery. During this intravenous antibiotic treatment he presented with symptoms of heart failure. The diagnosis of infective endocarditis of the mitral valve with mixed pathology and impaired LV function was established on day 26 of life. His weight was 4kg at that time. On day 34 of life attempt of valve repair failed and 17mm mechanical prosthesis was implanted into mitral position. 2 subsequent bypass surgeries followed this initial operation on day 79 and 82 due to partially blocked prosthesis including re-do mitral valve replacement. Despite otherwise good clinical progression conversion from iv Heparin to Warfarin remained unsuccessful requiring several courses of thrombolysis due to repeated blockage of one leaflet of the prosthesis. Treatment and Complication: The mechanical prosthesis was replaced with Melody valve 6 months after the initial and 4 months after the last surgery at a body weight of 7.4kg. The Melody valve was implanted by dilating it to 14mm under direct vision. Post bypass transoesophageal echocardiography showed a 4mm significant paravalvular leak (figure 1). Result No significant leak after repeat balloon dilatation of the valve was detected (figure 2).

Plasma atrial natriuretic peptide was measured in 13 children between the ages of 1 week and 2 years 9 months during atrioventricular re-entrant tachycardia and 15 minutes after the restoration of sinus rhythm. There was a significant decline in atrial natriuretic peptide during sinus rhythm. Plasma concentrations of the peptide were significantly higher during tachycardia in seven infants under 1 year than in the six older children. The heart rates and the duration of tachycardia were not significantly different in the two age groups. Cardiac failure was present in five of seven children under 18 weeks of age during tachycardia but in none of the older children. The plasma concentration of atrial natriuretic peptide did not significantly correlate with duration of tachycardia or heart rate. If tachycardia occurs in young infants the low functional reserve capacity of the developing heart leads to cardiac failure more frequently and it is likely that this was the cause of the significantly higher plasma concentration of atrial natriuretic peptide in the younger children.

Thirteen patients have undergone surgical treatment because of subaortic obstruction in hearts with a univentricular atrioventricular connection. Nine patients underwent surgical enlargement of the ventricular septal defect and four patients had construction of an aortopulmonary anastomosis and closure of the pulmonary trunk (the Damus-Kaye-Stansel procedure). Two patients undergoing enlargement of the septal defect and two having the Damus-Kaye-Stansel procedure also had a modified Fontan procedure. One patient had complete atrioventricular dissociation after direct enlargement of the ventricular septal defect, which necessitated insertion of an epicardial pacemaker. One patient died within 30 days of the operation after enlargement of the defect and two patients after the Damus-Kaye-Stansel procedure. There was one late death, occurring in a patient who underwent enlargement of the ventricular septal defect Ten patients have subsequently undergone conventional cardiac catheterization and angiography or transcutaneous Doppler flow studies to assess the relief of the subaortic obstruction. The result has been satisfactory in all. Because of this experience, we now recommend direct surgical enlargement of the restrictive ventricualr septal defect for direct relief of subaortic stenosis occurring with a univentricular atrioventricular connection to a dominant left ventricle, inasmuch as it appears to be hemodynamically effective with a low operative mortality and morbidity The Damus-Kaye-Stansel procedure can also have a role in relieving subaortic stenosis when the atria are connected to a dominant right ventricle.

Transposition of the great arteries is frequently complicated by the early onset of pulmonary vascular disease. It is difficult to measure pulmonary blood flow by the Fick principle because the pulmonary arteriovenous oxygen content difference is small and bronchial blood flow is increased in this condition. In eight patients (mean age 7.7 years, range 3 months to 29 years) with transposition of the great arteries mass spectrometry was used to measure oxygen uptake and predict pulmonary end capillary blood oxygen content. The effects of the bronchial circulation were studied by computer modelling. There was close agreement between pulmonary end capillary and pulmonary vein blood oxygen contents but the resultant percentage difference in arteriovenous content difference was significant (mean (SE of difference)) (14.5(3.8)%). The effect of the bronchial circulation was to give spuriously high estimates of pulmonary blood flow. The error was greatest when oxygen consumption was low and aortic blood was very desaturated.

Over a 5 year period open valvotomy was performed on 13 patients under the age of one year with critical aortic stenosis. All 13 survived operation. There were two late deaths--one 38 days after operation, associated with an unrelated neurosurgical procedure and the other 2 years 6 months after when aortic root enlargement and replacement of the aortic valve were performed. During this period two other infants presented with aortic stenosis. One, who was very ill before transfer, died before operation could be performed. The second patient had a hypoplastic left ventricle with a small mitral valve ring and was, therefore, considered to be part of a different subgroup. All the surviving children have been followed up (median length of follow up 2 years and 11 months, range 7 months-5 years). Left ventricular function, in terms of percentage systolic wall thickening, was shown to be significantly impaired in all age groups. Peak diastolic thinning was abnormal in those children aged from 3 to 5. The aortic valve gradient, as assessed by peak instantaneous continuous wave Doppler, was less than 40 mm Hg in five patients and between 40 and 70 mm Hg in seven patients. One patient, with appreciable restenosis, has undergone successful percutaneous balloon dilatation of the aortic valve.

Ventricular pressure-volume diagrams were obtained from the right ventricle in patients before and after relief of right ventricular pressure load, in patients with volume loaded right ventricles, and from the left ventricle in patients after the Mustard procedure for transposition of the great arteries. The patterns of ejection during pressure development and decline were similar in patients after relief of pressure load and in those with isolated volume load. A right ventricular pressure load, however, reduced ejection during the two "isovolumic" periods, and the overall shape of the pressure-volume loop resembled that of the normal left ventricle. Pressure-volume diagrams obtained from the left ventricle after the Mustard procedure were indistinguishable from the normal right ventricle, which accords with the hypothesis that the normal right ventricular contraction pattern is a consequence of loading conditions rather than a reflection of an intrinsic property of the myocardium.

ObjectivesThe purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.BackgroundPulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.MethodsPredictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004. We also assessed the predictive value of coronary fistulae and right atrial pressure (RAP) score (comprising the tricuspid valve, foramen ovale, and ductus venosus Doppler).ResultsOne-half of the cohort was first assessed before 23 gestational weeks (range 15.7 to 33.7 weeks). The TV z-score was a good predictor at all gestations, but the best predictive scores for specific gestations were pulmonary valve (PV) z-score (31 weeks). The RAP score and coronary fistulae were good independent predictors: RAP score >3 predicted BV with area under the curve of 0.833, and detection of fistulae usually predicted a UV route.ConclusionsThe best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.

Objectives:Pediatric cardiology has an expanding role in fetal and pediatric screening. The aims of this study were to observe how district hospitals use a pediatric telecardiology service, and to compare the costs and outcomes of patients referred to specialists by means of this service or conventionally.Methods:A telemedicine service was set up between a pediatric cardiac center in London and four district hospitals for referrals of second trimester women, newborn babies, and older children. Clinicians in each hospital decided on the role for their service. Clinical events were audited prospectively and costed, and patient surveys were conducted.Results:The hospitals differed in their selection of patient groups for the service. In all, 117 telemedicine patients were compared with 387 patients seen in London or in outreach clinics. Patients selected for telemedicine were generally healthier. For all patients, the mean cost for the initial consultation was £411 for tele-referrals and £277 for conventional referrals, a nonsignificant difference. Teleconsultations for women and children were significantly more expensive because of technology costs, whereas for babies, ambulance transfers were much more costly. After 6-months follow-up, the difference between referral methods for all patients was nonsignificant (telemedicine, £3,350; conventional referrals, £2,172), and nonsignificant within the patient groups.Conclusions:Telemedicine was perceived by cardiologists, district clinicians, and families as reliable and efficient. The equivocal 6-month cost results indicate that investment in the technology is warranted to enhance pediatric and perinatal cardiology services.

A full-term neonate presented with systemic arterial hypoxia at 12 hours of age. Examination revealed a single second heart sound preceded by a holosystolic murmur. The ECG and chest X-ray demonstrated right atrial enlargement. Cross-sectional echocardiography demonstrated severe right ventricular hypertrophy and mild high-velocity tricuspid regurgitation. The pulmonary valve was normally formed and mobile, with a membrane connecting the leaflet tips, creating functional atresia (Figure 1 and Figure 2⇓). At cardiac catheterization, the diagnosis was …

Two cases of juxtaductal aortic atresia diagnosed as coarctation on clinical and Doppler echocardiographic grounds are presented. The misleading nature of the Doppler flow velocity characteristics in this condition is discussed and raises questions as to the source of these flow velocities in coarctation.

The cross-sectional echocardiographic and postmortem appearances of the heart from a patient with the rare association of aortic valve atresia, aortopulmonary window, and interrupted aortic arch are described. Differentiation of this anomaly from truncus arteriosus with interrupted aortic arch is important.

The morphology of the various types of interatrial communications has been known since the early description by Rokitansky,1 but the clinical diagnosis was not described until 1941.2 Hospital mortality after surgical repair of atrial septal defects during the early years was about 3%3 and for many years it has been less than 1%, with correspondingly low complication rates. The era of transcatheter closure of secundum atrial septal defects is now well established but confusion reigns regarding ideal occlusion devices and indications for their use. The past and current success of surgery cannot be ignored when evaluating current fashions, and although it is true that routine closure is not of proved benefit to all patients there is a general consensus among cardiologists and surgeons that when an atrial septal defect gives rise to right ventricular dilatation it should be closed. Such defects usually measure 10 mm or more in diameter and occupy one third or more of the length of the atrial septum in echocardiographic four chamber sections. Because most defects are closed, we are unlikely ever to know the true natural history of patients with an atrial septal defect. The most detailed study available was that of Campbell in 1970,4which was based on a highly selected group of patients with large and clinically obvious defects in whom actuarial survival to the age of 60 was approximately 15%, whereas in the general population it is about 85%. Few would doubt that smaller defects carry a much better prognosis. Many aspects of the natural history are well known4 5 and in general terms closure of these defects does benefit most patients.6 Survival when surgical repair is done during the first few years of life is the same as that of a matched general population. Older age …