Background— To better define determinants of mortality in patients with univentricular physiology, a database registry was created born 1985 or earlier Fontan surgery who were followed up at Children’s Hospital Boston. Methods and Results— A total 261 patients, 121 whom (46.4%) women, had first median age 7.9 years: right atrium–to–pulmonary artery connection 135 (51.7%); atrium to ventricle 25 (9.6%); cavopulmonary 101 (38.7%). Over 12.2 years, 76 (29.1%) died, 5 (1.9%) cardiac...

Background— Transcatheter pulmonary valve placement is an emerging therapy for regurgitation and right ventricular outflow tract obstruction in selected patients. The Melody was recently approved the United States dysfunctional conduits. Methods Results— From January 2007 to August 2009, 136 patients (median age, 19 years) underwent catheterization intended implantation at 5 centers. Implantation attempted 124 patients; other 12, transcatheter not because of risk coronary artery compression...

BACKGROUND Balloon expandable intravascular stents have been used to support vessel walls in coronary and peripheral arteries adults. The purpose of this study was examine the efficacy safety these treatment congenital heart disease. METHODS AND RESULTS Forty-five were placed 30 patients, who 0.2-30.2 years old (weight, 3.5-76 kg). Patients with areas stenosis that difficult approach surgically chosen. Stents mounted over balloons by standard catheterization techniques. Twenty-three patients...

Between January and October, 1987, we attempted percutaneous transcatheter closure of seven ventricular septal defects (VSD) in six patients; none the patients was a candidate for operative management. Patients' ages ranged from 8 months to 82 years (6.0-70 kg); diagnoses included postinfarction VSD (n = 4), congenital 1), postoperative 2). Indications were shock or respiratory failure 5) multiple episodes endocarditis 1). Closure with Rashkind double umbrella: VSDs crossed via left...

Although pulmonary thromboendarterectomy is increasingly successful for the definitive treatment of chronic thromboembolic hypertension (CTEPH), not all patients have surgically accessible disease. Others are poor surgical candidates because comorbid illness. Therefore, selected patients, we defined and implemented an alternative interventional strategy balloon angioplasty (BPA).Eighteen (mean age, 51.8 years; range, 14 to 75 years) with CTEPH underwent BPA; they averaged 2.6 procedures...

Factors associated with impaired clinical status in a cross-sectional study of patients repaired tetralogy Fallot (TOF) have been reported previously.To determine independent predictors major adverse outcomes late after TOF repair the same cohort during follow-up evaluated by cardiac magnetic resonance (CMR).Clinical at latest was ascertained 88 (median time from to baseline evaluation 20.7 years; median most recent 4.2 years). Major included (a) death; (b) sustained ventricular tachycardia;...

Ventricular dysfunction, elevated pulmonary vascular resistance, and residual distal artery distortion contribute to early mortality after a Fontan operation; they may be transient or reversible. A baffle fenestration, allowing right-to-left shunting, maintains cardiac output limits right atrial pressure. fenestration was surgically created at the time of modified repair in 20 consecutive patients. Risk factors included pressure 18 mm Hg more, end-diastolic 12 valvar regurgitation,...

We attempted percutaneous transcatheter-balloon mitral commissurotomy in eight children and young adults (9 to 23 years of age) with rheumatic stenosis. The atrial septum was traversed by needle puncture, an 8-mm angioplasty balloon advanced over a guide wire. septal perforation then dilated allow passage the valvuloplasty catheter (18 25 mm) across annulus. Inflation transmitral decreased end-diastolic gradient temporarily all patients (from 21.2 +/- 4.0 mm Hg [mean S.D.] 10.1 5.5 Hg; P...

Preventing the progression of fetal aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS) requires identification fetuses with salvageable hearts who would progress HLHS if untreated, a successful in utero valvotomy, and demonstration that valvotomy promotes growth utero. Fetuses meeting first criterion are undefined, previous reports AS dilation have not evaluated impact intervention on structures.We offered 24 mothers whose had AS. At least 3 echocardiographers assigned high...

Background— Fetal aortic valvuloplasty may prevent progression of stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential optimize patient selection for fetal intervention. The aim this study was define echocardiographic features associated midgestation HLHS. Methods and Results— echocardiograms were reviewed from 43 diagnosed normal ventricular (LV) length at ≤30 weeks’ gestation. Of 23 live-born patients available follow-up...

Balloon dilation angioplasty (BDA) was attempted nine times in eight infants and children with aortic coarctation. In three (all associated ventricular septal defect or atrioventricular canal marked hemodynamic instability) at a site of narrowing that had not been operated on previously. Although the coarctation gradient fell 40% more over short term two three, there no angiographic late evidence improvement. All underwent subsequent surgery. Five dilations were performed four who previously...

Aortic stenosis in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic heart syndrome when associated certain physiological findings. Prenatal balloon aortic valvuloplasty may improve growth and function, possibly preventing evolution syndrome.Between March 2000 October 2008, 70 fetuses underwent attempted for critical evolving syndrome. We analyzed this experience determine factors procedural postnatal outcome. The median gestational...

Background— Among patients undergoing transcatheter pulmonary valve (TPV) replacement with the Melody valve, risk factors for stent fracture (MSF) and right ventricular outflow tract (RVOT) reintervention have not been well defined. Methods Results— From January 2007 to 2010, 150 (median age, 19 years) underwent TPV implantation in Investigational Device Exemption trial. Existing conduit stents from a prior catheterization were present 37 (25%, fractured 12); 1 or more new prestents placed...

Background— The Melody transcatheter pulmonary valve (TPV) was approved for implantation in obstructed right ventricular outflow tract conduits 2010 after a multicenter trial demonstrating improvements conduit obstruction, regurgitation, and pressure. A recognized risk contraindication to TPV is the demonstration of coronary artery (CA) compression during balloon angioplasty or stent placement overlying conduit. This study first characterize CA this population. Methods Results— From 2007...

Fetal aortic valvuloplasty can be performed for severe midgestation stenosis in an attempt to prevent progression hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal valvuloplasty. The postnatal outcomes and survival the BV patients, comparison with those managed as HLHS, have not been reported.We included 100 who underwent evolving HLHS from March 2000 January 2013. Patients were categorized based on management or HLHS....

Background Pulmonary arteriovenous malformations (PAVMs) are a known complication after some types of cavopulmonary anastomoses (CVPAs). Their cause is unknown, but they may be related to the absence pulsatile flow or presence circulating factors. These PAVMs diffuse and presumed progressive irreversible. Methods Results All patients with congenital heart disease (CHD) seen at Children’s Hospital, Boston, Mass, between 1970 1993 were reviewed. We report on 10 CHD who found have developed...

Balloon-expandable stents (Johnson and Johnson Interventional Systems) have been in use for congenital heart disease since late 1989. They made possible treatment previously untreatable branch pulmonary artery stenoses systemic venous stenosis. The purpose of this report is to detail the results intermediate-term follow-up used disease.Eighty-five patients underwent placement 121 Houston Boston. Fifty-eight had put arteries, nine conduits or outflow tracts, 21 narrowed Fontan anastomoses....

BACKGROUND Ectopic atrial tachycardia (EAT) is a reversible cause of cardiomyopathy but may be quite difficult to control with conventional therapy. Transcatheter ablation radiofrequency current was tested as an alternative medical or surgical treatment this condition. METHODS AND RESULTS Twelve young patients (aged 10 months 19 years) drug-resistant EAT were treated direct transcatheter the ectopic focus using (RF) energy. All had depressed left ventricular contractility by...

Forty patients were catheterized for closure of atrial septal defects with the Rashkind patent ductus arteriosus umbrella device, a modified and newly designed Lock Clamshell Occluder. Patients weighed 8 kg or more (a requirement transvenous access 11F delivery sheath) had suitable based on two-dimensional echocardiography. The new device was at least 1.6 times diameter defect as determined by balloon sizing catheterization. followed up telephone, clinical examination, echocardiography 6...

To examine the impact of hospital caseload on in-hospital mortality for pediatric congenital heart surgery.Population-based, retrospective cohort study.Acute care hospitals in California and Massachusetts.Children undergoing surgery disease, identified by presence procedure codes indicating surgical repair a defect computerized statewide discharge abstract databases. Cases were grouped into four categories based complexity procedure.Adjusted odds ratios (OR) death estimated using generalized...