Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation.

OBJECTIVE: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO). DESIGN: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an "open comment period." Final approval of each QM was provided by a vote of the ACC ACPC Council. PATIENTS: Patients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded. RESULTS: Twelve candidate QMs were generated. Seven metrics passed the RAND-UCLA process. Four passed the "open comment period" and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age. CONCLUSIONS: Application of the RAND-UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.

Neonatal acute myocardial infarction of unknown etiology treated with surgical thrombectomy.

Coronary artery thrombosis and resultant myocardial infarction in neonates are exceedingly rare. Seldom does a neonate survive this myocardial insult. Often there is an underlying predisposition to coronary artery thrombosis, such as thrombophilia, central line placement, or myocarditis. Treatment attempts have consisted of thrombolysis and supportive care. We present a case of acute left main coronary artery (LMCA) thrombosis of unknown etiology in a neonate, without ventricular dilatation. Flow into the LMCA was suggested echocardiographically, but a high clinical suspicion led to urgent cardiac catheterization. Once complete occlusion of the LMCA was demonstrated angiographically, emergent surgical thrombectomy was performed, which has never previously been reported.

Streptococcus pneumoniae causing mycotic aneurysm in a pediatric patient with coarctation of the aorta.

Mycotic aneurysms are rare in patients with congenital heart disease, but may occur in those with aortic coarctation and abnormal aortic valve. Rapid diagnosis of mycotic aneurysm is of extreme importance given the significant reported incidence of morbidity and mortality across all age groups. Aortic aneurysm is uncommon before the second decade of life, and here we report a 10-year-old male patient with new diagnosis of aortic coarctation and bicuspid aortic valve, who developed a rapidly enlarging mycotic aneurysm from Streptococcus pneumoniae. Cardiac magnetic resonance imaging was crucial in making the diagnosis, as well as in follow-up.

Atrial tachyarrhythmias in neonatal enterovirus myocarditis.

Enterovirus myocarditis can be a serious infection in neonates, complicated by cardiac dysfunction and arrhythmias. Although decreased systolic function and ventricular arrhythmias have been reported, there is limited description of significant atrial involvement. We present an unusual series of three infants at our institution with enterovirus myocarditis in the neonatal period associated with atrial arrhythmias and dilation.

Persistence of bilateral arterial ducts in pulmonary atresia despite confluent branch pulmonary arteries: opportunity for two percutaneous therapeutic alternatives.

We present a case of a newborn infant with double inlet left ventricle, pulmonary atresia, confluent pulmonary arteries, and bilateral arterial ducts (AD), to discuss the therapeutic alternatives offered by interventional catheterization techniques in this anatomic arrangement. The infant initially underwent stenting of the right AD to stabilize pulmonary blood flow off of prostaglandin infusion. Three weeks later, she developed left pulmonary artery isolation upon closure of the left arterial duct. An additional stent was placed in the pulmonary artery confluence, restoring blood flow to the left lung and significantly improving her oxygen saturations. At 6 months of age she underwent her first surgical procedure, a successful bidirectional cavopulmonary anastomosis with removal of the left pulmonary artery stent and patch enlargement of the pulmonary artery confluence. She continues to do well in clinical follow-up at 16 months of age.

Transcatheter occlusion of a residual muscular ventricular septal defect using an Amplatzer duct occluder in a child with congenitally corrected transposition of the great arteries.

Transcatheter occlusion has become an acceptable alternative to surgery in patients with congenital muscular and residual post-surgical ventricular septal defects (VSD). We present a case of an 11 year old male with congenitally corrected transposition of the great arteries, dextrocardia, pulmonary atresia, VSD, and advanced second degree atrioventricular block who underwent successful transcatheter occlusion of a residual post-surgical VSD with an Amplatzer duct occluder, in preparation for transvenous pacemaker implantation.

Impact of transcatheter occlusion of a patent ductus arteriosus on atrial septal defect diameter in children.

BACKGROUND: A persistent patent ductus arteriosus (PDA) may delay closure of a coexisting atrial septal defect (ASD) due to volume loading and enlargement of the left atrium. The purpose of this study was to investigate the natural history of ASD size in patients with a PDA following transcatheter PDA occlusion. METHODS: All patients with an ASD and a PDA who underwent transcatheter PDA occlusion at Texas Children's Hospital were identified. Patients with ASD diameter <3 mm, or additional cardiac defects were excluded. Eight patients (7 females) with small- to moderate-sized ASDs and a PDA were identified. Patient demographics, echocardiographic data, and cardiac catheterization data were recorded. Data were analyzed by 1-tailed t-test. RESULTS: Following PDA occlusion, ASD diameter decreased in 6 of 8 patients by a mean of 3.8 mm (+/-2.3 mm), including 2 that closed. The median duration of follow-up was 689 days. One ASD remained unchanged and 1 increased in size. The mean maximum ASD diameter decreased from 6.4 mm (+/-2.2 mm) to 3.9 mm (+/-3.4 mm) (P = .03). Two patients underwent subsequent transcatheter ASD occlusion. CONCLUSION: Following transcatheter PDA occlusion, small- to moderate-sized ASDs have significant probability to decrease in size, and possibly close. In infants and children, we recommend transcatheter PDA occlusion, and serial follow-up of the size of the ASD. This will allow many small- to moderate-sized ASDs to either close, or become smaller, obviating the need for future intervention.

Atrial septal stent implant: atrial septal defect creation in the management of complex congenital heart defects in infants.

OBJECTIVES: Certain congenital heart defects require the creation of an unrestrictive atrial septal defect (ASD) to relieve atrial hypertension, to maintain systemic cardiac output, or to achieve adequate atrial mixing to improve systemic oxygen saturation. We describe a series of patients, ranging in age from 5 weeks to 17 months, in whom we implanted a stent across the atrial septum to create a lasting, unrestrictive interatrial communication. METHODS: Five patients with left heart obstructive defects presented with severe left atrial hypertension. The patients weighed 3.5-10.1 kg. Patients 1 and 2 presented at 5 and 9 weeks of age, respectively. Patient 1 had a restrictive patent foramen ovale, and patient 2 had an intact atrial septum. Patients 3, 4, and 5 had previous cardiac surgery including atrial septectomy. At presentation, all patients were poor surgical candidates. RESULTS: Using standard percutaneous technique, a premounted Palmaz-Genesis stent (Cordis Corporation) was implanted across the atrial septum in all 5 patients, using balloons ranging from 7 to 10 mm diameter. Each patient had a dramatic decrease in left atrial pressure and increased ASD diameter. All stented ASDs remained widely patent, confirmed by Doppler echocardiography, until elective surgical stent explant. CONCLUSION: Patients presenting beyond the neonatal period with left heart obstructive defects and intact atrial septum or restrictive ASD present a challenging problem. We report a safe and effective technique for relief of left atrial hypertension in infants using premounted intravascular stents. All patients had immediate marked hemodynamic improvement.

Transcatheter atrial septal defect closure: modified balloon sizing technique to avoid overstretching the defect and oversizing the Amplatzer septal occluder.

The objective of this study was to evaluate a new technique of sizing atrial septal defects (ASDs) for transcatheter device closure. ASD closure using the Amplatzer septal occluder (ASO) device is commonly performed. Complications, including arrhythmias, pericardial effusions, and perforations, may be related to oversizing ASDs and choosing larger devices. Two methods were used to size ASDs using a compliant balloon. In some patients, the balloon was inflated until a waist was visible [(+)waist]; in others, only until no shunting was demonstrable by echocardiogram [echo; (-)waist]. The device was selected and implanted using standard procedure and echo guidance. One hundred seventeen patients underwent secundum ASD closure with an ASO device. There were 43 patients in the (-)waist group and 74 in the (+)waist group. All devices were implanted successfully. The initial echo ASD diameter was larger in the (-)waist group compared to the (+)waist group (P = 0.01). There was a smaller difference between the initial echo and balloon-sized ASD diameters in the (-)waist group (P < 0.02). ASO device size implanted (in mm greater than echo ASD diameter) was smaller in the (-)waist group (P < 0.01). There were 0/43 complications in the (-)waist group and 5/74 in the (+)waist group. The complete closure rate was the same in both groups. Sizing an ASD by inflating a compliant balloon just until shunting is eliminated, and not until a waist is visible, results in less overstretching of the ASD and selection of a smaller ASO device, achieving similar closure rates and potentially fewer complications.

Use of cutting balloon for palliative treatment in tetralogy of Fallot.

In tetralogy of Fallot, severe cyanosis due to insufficient pulmonary blood flow necessitates early intervention. The Cutting Balloon, developed for percutaneous coronary angioplasty, was used successfully for transcatheter incision and dilation of infundibular and valvar pulmonary stenosis in four patients with tetralogy of Fallot.