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Some patients with pulmonary valve stenosis do not respond to balloon valvuloplasty and must undergo surgical repair. We report the case of a 12-year-old child with pulmonary valve stenosis and Noonan syndrome in whom we performed transcatheter Melody pulmonary valve implantation after balloon dilation failed. The result was excellent. This technique can be proposed as an alternative to surgery in such cases.

Transcatheter Pulmonary Valve Implantation (TPVI) has emerged as an attractive therapeutic option for the treatment of right ventricular outflow tract dysfunction. By 2015, more than 7000 transcatheter pulmonary valve implantation have been performed. Two valves are currently used for TPVI. The Melody Valve (MeV) (Medtronic Inc., Minneapolis, Minnesota), which can be expanded to 22 mm diameter. The Edward Sapien Valve (ESV) (Edwards Lifesciences LLC, Irvine, California), which can be expanded to 26 mm diameter. This chapter summarizes the concept, its evolution in clinical practice, current applications, and the devices in current use. Early and mid-term outcomes are presented as well as projected long term results and future developments.

A 24-year-old woman presented with a recent increase in dyspnea on exertion and development of presyncope. The patient stated that she has reproducible episodes of dizziness and near fainting when she climbs a flight of stairs and activity is limited to a slow gait.

Thrombosis in children with dilated and inflammatory cardiomyopathy is an unpredictable complication with potentially important morbidity.To determine the prevalence, associated factors, management and outcomes of thrombosis in this setting.Data were obtained from review of medical records. Factors associated with thrombosis and the impact on outcome were sought.From 1990 to 1998, 66 patients that presented with dilated cardiomyopathy were followed for a median interval of 1.4 years (range 0 to 9.79 years) from first presentation. Thrombosis was diagnosed in four patients at presentation and in four patients during follow-up. Thrombosis was noted in one additional patient at examination after death. The overall nine-year period prevalence of thrombosis was 14%. Anticoagulation was started at presentation in 31% of patients. The mean left ventricular ejection fraction at presentation was significantly lower in those given anticoagulation (19+/-8%) versus those who were not (32+/-15%; P0.001). The mean ejection fraction at presentation was similar in those patients with (25+/-10%) versus those without thrombosis (28+/-15%; P = 0.44). During follow-up, 11 patients died and seven underwent cardiac transplantation. Kaplan-Meier estimates of freedom from death or transplantation were 88% at three months, 81% at one year and 70% at five years. Survival free of transplantation was not affected by thrombosis.Thrombosis is common in children with cardiomyopathy, can occur at any time in the patients' clinical course and is not related to clinical features or survival free of transplantation. The relevance and prevention of thrombosis in this setting remains unclear.

Background There are no large series describing the morphologic spectrum and the clinical outcomes of children with ductal origin of the distal pulmonary artery (PA). Methods Medical records were reviewed for all children presenting between 1970 and 2001. Angiograms were reviewed at presentation and at last available follow-up. Results Forty-five patients were identified with median presenting age of 14 days (range, birth to 6.5 years). Ductal origin of the distal PA occurred as an isolated finding in 16 patients (36%), with tetralogy of Fallot in 12 (27%), with pulmonary atresia–ventricular septal defect in 13 (29%), and with heterotaxy in 4 (9%). Diagnostic pulmonary venous wedge angiography was performed in 21 patients (47%). Surgical procedures were undertaken in 31 patients, and were initial systemic-ductal PA shunt in 13 patients, interposition graft in 6, direct anastomosis to the main PA in 2, ductal PA banding in 2, unifocalization of the ductal PA with complete or staged pulmonary atresia-ventricular septal defect repair in 7, and heart transplantation in 1 patient. Surgical revision was required in 3 patients and catheter interventions in 12 patients. Overall 20-year survival was 70% and was improved among patients without congestive heart failure at presentation ( p = 0.08, hazard ratio: 2.81). Reconstruction of the ductal PA decreased the prevalence of pulmonary parenchymal hypoplasia ( p Conclusions Ductal origin of the distal PA is associated with important multisystem morbidity and mortality. Early diagnosis and repair of the ductal PA, especially in children presenting with pulmonary overcirculation, may improve outcomes.

An aortopulmonary window is a communication between the ascending aorta and pulmonary trunk in the presence of two separate arterial valves, and is often complicated by other associated defects. We sought to determine management and related outcomes in patients with this malformation.We identified those patients presenting between 1969 and 1999 from the databases held in our Departments of Cardiology, Pathology and Cardiovascular Surgery. We obtained data relating to issues concerning demography, clinical findings, imaging, management and outcome.The median age at presentation for the 42 patients identified, of whom 23 were female, was 62 days, with a range from birth to 6 years. Associated cardiac defects were present in 34 patients, including interruption of the aortic arch in 6 patients. The correct diagnosis was initially missed in 13 patients. Of the patients, six died without surgical repair, and 1 patient was lost-to-follow-up. Repair was performed in 35 patients, subsequent to repair of other defects in 4, in association with repair of other defects in 17, of whom 3 died, and as an isolated procedure in 14 patients, one of the latter being treated by transcatheter closure. Overall, there were 9 deaths, all in patients with complex associated defects, except 1 patient with a missed aortopulmonary window after repair of aortic coarctation. Kaplan-Meier estimates of survival were 81% at 3 months until 11.5 years, and 69% up to 21 years. Only the presence of interrupted aortic arch was independently associated with increased time-related mortality, the hazard ratio being 5.87 (p = 0.009).The outcome for an isolated lesion is excellent. Mortality occurs mainly before repair, mostly with complex associated lesions, particularly interruption of the aortic arch.

A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.

Primary diffuse congenital pulmonary vein stenosis (PDPVS) is a severe condition with poor prognosis. No therapeutic strategy has been found to be effective in its management to date. We report the case of a premature child with severe PDPVS who underwent multiple transcatheter interventions as bridge to heart-lung transplantation.

SummaryBackgroundVentricular septal defect (VSD) after acute myocardial infarction is a catastrophic event.AimsWe describe our multicentre experience of a defect closure strategy that combined surgery and transcatheter closure.MethodsData were obtained by retrospective chart review.ResultsTwenty patients (mean age, 67years) from three centres were studied. Median time from myocardial infarction to VSD was 6 (range, 3–9) days. Acute cardiogenic shock occurred in 12 (60%) patients. Median defect diameter by echocardiography was 18 (range, 12–28) mm. Median time to first surgical or percutaneous closure was 18 (range, 4–96) days. Twenty-seven procedures were performed in the 20 patients. Surgical closure was undertaken in 14 patients and contraindicated in eight, six of whom underwent percutaneous closure; the other two, after reconsideration, proceeded to surgical closure. No procedural complications occurred with percutaneous closure. Percutaneous closure patients were older than surgical patients (75 vs. 64years; P=0.01) and had a higher mean logistic EuroSCORE (87% vs. 67%; P=0.02). Rates of residual shunt and mortality did not differ between surgical and percutaneous patients (P=0.12 and 0.3, respectively). Those who underwent early VSD closure (

Kawasaki disease (KD) is a self-limited vasculitis that can have significant morbidity and even mortality if not identified and treated early. The purpose of this case study is to highlight the importance of keeping KD in the differential diagnosis, even if all clinical criteria are not met. This is especially true in children younger than 1 year of age, who are more likely to have an incomplete or atypical presentation. The patient in this study is a 12-week-old African-American male with no significant past medical history who presented with a 10-day history of fever up to 105° F. An echocardiogram shortly after admission demonstrated normal cardiac structure and function with evidence of coronary artery abnormalities. Diagnosis of KD (treatment with intravenous immunoglobulin, aspirin, and a tumor necrosis factor-alpha inhibitor) and follow-up imaging are detailed. Treatment of KD in the acute phase is aimed at preventing coronary thrombosis and aneurysm formation.

Transcatheter closure has become an accepted alternative to surgical repair for ostium secundum atrial septal defects (ASD). However, large ASDs (>38 mm) and defects with deficient rims are usually not offered transcatheter closure but are referred for surgical closure. Several studies have reported the feasibility of transcatheter closure in complex cases with a variety of modified implantation methods such as balloon assisted technique (BAT). AA Pillai and co-authors report the transcatheter closure of ASD ≥35 mm with the BAT. However, the true significance of their study is rather in demonstrating the superiority of BAT to conventional technique and other modified implantation techniques in patients with ASD rather than feasiblity of transcatheter closure of large defect. Finally, a single dimension does not reflect the true ASD size because many defects are not round in shape but rather oval or even crescentric. Hence, future studies will need not only to demonstrate the ideal implantation method but also the appropriate 3-dimensional (3D) imaging definition of the defect in this patient population.

In the last 15 years the development of catheter-directed percutaneous therapies have improved the delivery of care to children with congenital heart lesions. Paralleling these advances, enhanced surgical techniques are now applied to complex cardiac lesions previously thought to be inoperable. This chapter outlines several treatment strategies which utilize surgical and catheter-based algorithms to address congenital heart disorders.

Endomyocardial biopsy (EMB) is usually performed from the femoral veins in infants and small children. This requires the use of a long vascular sheath to direct a bioptome to the ventricular septum. We describe a technique of EMB in this population that obviates the need for the use of a long vascular sheath. A 5 Fr 0.038" internal diameter, no-sideholes Cobra catheter (Cook, Bloomington, IN) introduced via a 5 Fr short sheath was used to direct a 3 Fr, 60-cm-length Cook bioptome to the ventricular septum. From January 1998 to August 1999, this technique was used 22 times in seven patients for EMB (median, age 13 months; range, 1-72 months). Indications for EMB in this group were posttransplantation surveillance (21 times) and evaluation for cardiomyopathy/myocarditis (1). Median number of EMB specimens obtained per procedure was 6 (range, 5-9). There was one pneumopericardium seen post-EMB with this technique without hemodynamic embarrassment. EMB in infants and small children can be performed successfully, safely, and repeatedly from the femoral veins without the need for a long sheath.

Rationale: Closure of the ductus arteriosus (DA) is essential for the transition from fetal to neonatal patterns of circulation. Initial P O 2 -dependent vasoconstriction causes functional DA closure within minutes. Within days a fibrogenic, proliferative mechanism causes anatomic closure. Though modulated by endothelial-derived vasodilators and constrictors, O 2 sensing is intrinsic to ductal smooth muscle cells and oxygen-induced DA constriction persists in the absence of endothelium, endothelin, and cyclooxygenase mediators. O 2 increases mitochondrial-derived H 2 O 2 , which constricts ductal smooth muscle cells by raising intracellular calcium and activating rho kinase. However, the mechanism by which oxygen changes mitochondrial function is unknown. Objective: The purpose of this study was to determine whether mitochondrial fission is crucial for O 2 -induced DA constriction and closure. Methods and Results: Using DA harvested from 30 term infants during correction of congenital heart disease, as well as DA from term rabbits, we demonstrate that mitochondrial fission is crucial for O 2 -induced constriction and closure. O 2 rapidly (2 O 2 production. Subsequently, fission increases complex I activity. Mitochondrial-targeted catalase overexpression eliminates P O 2 -induced increases in mitochondrial-derived H 2 O 2 and cytosolic calcium. The small molecule Drp1 inhibitor, Mdivi-1, and siDRP1 yield concordant results, inhibiting O 2 -induced constriction (without altering the response to phenylephrine or KCl) and preventing O 2 -induced increases in oxidative metabolism, cytosolic calcium, and ductal smooth muscle cells proliferation. Prolonged Drp1 inhibition reduces DA closure in a tissue culture model. Conclusions: Mitochondrial fission is an obligatory, early step in mammalian O 2 sensing and offers a promising target for modulating DA patency.

A 5-year-old male presented at birth with tachypnea and abnormal chest x-ray. He was diagnosed with Scimitar Syndrome in the neonatal period by ultrasound, which revealed partial anomalous pulmonary venous return (PAPVR) of the right pulmonary vein to the inferior vena cava, as well as dextroversion of the heart, hypoplastic right pulmonary artery, and right lung hypoplasia. Due to the large shunt volume, the patient exhibited signs of congestive heart failure with dilatation of the left atrium and left ventricle. The patient underwent cardiac catheterization, which confirmed the presence of PAPVR as well as a large aorto-pulmonary collateral connecting the sequester to the descending aorta. Due to the high flow within the sequester artery a combined "sandwich" technique was used with two AGA vascular plugs (II) and MWCE Tornado coils. Complete closure of the collateral was achieved.

Objectives:We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998.Background:Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair.Methods:We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports.Results:The mean initial Z-score for the diameter of the tricuspid valve was −0.99 ± 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up.Conclusions:The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Peripheral pulmonary artery stenosis challenges therapeutic algorithms for the management of congenital heart malformations. Surgical repair of the proximal pulmonary artery lesion remains with a high recurrence rate while the distal lesions are difficult to access. With the development of transcatheter interventional strategies in the early 1980s, a number of transcatheter treatment options became available. In this review, we summarize the current state of the art for interventional strategies in the management of peripheral pulmonary artery stenosis.

Knowledge of normal aortic dimensions is important in the management of children with aortic disease. So as to define such dimensions, we undertook a retrospective review of clinical data and aortic cineangiograms from 167 subjects without aortic disease having a mean age of 3.67 years, with a range from 0.01 to 14.95 years. Amongst the patients, 56 were without detectable cardiac lesions, 66 patients had mild pulmonary stenosis, 30 were seen with Kawasaki disease, and 15 with small interatrial defects within the oval fossa. Aorto-grams were available in all. No patient had any hemodynamic derangement that would have affected the aorta during intrauterine life or childhood. Systolic dimensions were measured in the ascending and descending aorta at the level of the carina, at the transverse aortic arch distal to the brachiocephalic, of the left common carotid artery at its origin, at the transverse aortic arch distal to the left common carotid artery, at the aortic isthmus, and of the aorta at the level of the diaphragm. A regression analysis model was used to establish the range of predicted normal values, with their confidence limits, standardizing the values to height as the biophysical parameter having the highest correlation to aortic dimensions. Normal ranges were established for all the levels of measurement. The data should prove useful in identifying abnormalities of the thoracic aorta during childhood, and when assessing the outcomes of interventions.

Septoplasty of the atrial septum was performed with sequential balloon dilation following radiofrequency-assisted perforation of an intact atrial septum in two newborn infants with hypoplastic left heart syndrome and one with double-outlet right ventricle.