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.

Comparison of plasma B-type natriuretic peptide levels in single ventricle patients with systemic ventricle heart failure versus isolated cavopulmonary failure.

The measurement of plasma B-type natriuretic peptide (BNP) has emerged as a useful biomarker of heart failure in patients with cardiomyopathy. The pathophysiology of heart failure in single ventricle (SV) circulation may be distinct from that of cardiomyopathies. A distinct pattern of BNP elevation in heart failure in the SV population was hypothesized: it is elevated in heart failure secondary to ventricular dysfunction but not in isolated cavopulmonary failure. BNP was measured prospectively in SV patients at catheterization (n = 22) and when assessing for heart failure (n = 11) (7 normal controls). Of 33 SV subjects (median age 62 months), 13 had aortopulmonary connections and 20 had cavopulmonary connections. Median and mean +/- SD BNP levels by shunt type were 184 and 754 +/- 1,086 pg/ml in the patients with aortopulmonary connections, 38 and 169 +/- 251 pg/ml in the patients with cavopulmonary connections, and 10 and 11 +/- 5 pg/ml in normal controls, respectively (p = 0.004). Median systemic ventricular end-diastolic pressure (8mm Hg, R = 0.45), mean pulmonary artery pressure (14.5 mm Hg, R = 0.62), and mean right atrial pressure (6.5 mm Hg, R = 0.54) were correlated with plasma BNP. SV subjects with symptomatic heart failure from dysfunctional systemic ventricles had median and mean +/- SD BNP levels of 378 and 714 +/- 912 pg/ml (n = 18) compared with patients with isolated failed Glenn or Fontan connections (19 and 23 +/- 16 pg/ml [n = 7, p = 0.001]) and those with no heart failure (22 and 22 +/- 12 pg/ml [n = 8, p = 0.001]). Excluding the group with cavopulmonary failure, the severity of heart failure from systemic ventricular dysfunction was associated with plasma BNP. In conclusion, plasma BNP is elevated in SV patients with systemic ventricular or left-sided cardiac failure. BNP is not elevated in patients missing a pulmonary ventricle with isolated cavopulmonary failure.

The use of veno-venous extracorporeal membrane oxygenation for perinatal support of an infant with d-transposition of the great arteries, intact atrial and ventricular septa, and flow-restricted ductus arteriosus.

Prenatal assessment of a fetus with D-transposition of the great arteries demonstrated an absence of mixing between systemic and pulmonary circulations, and predicted lethal postnatal hypoxemia. A multidisciplinary meeting evaluated therapeutic options. After cesarean delivery, veno-venous extracorporeal membrane oxygenation was instituted in preparation for open atrial septectomy. The infant subsequently underwent an arterial switch procedure. Prenatal delineation of pulmonary and systemic circulations in the fetus with D-transposition of the great arteries influences postnatal management. Multidisciplinary planning enhanced the perinatal outcome.

Restrictive hemodynamics are present at the time of diagnosis of allograft coronary artery disease in children.

Clinical recognition of allograft coronary artery disease (ACAD) is challenging. We examined whether right heart hemodynamics can aid its diagnosis in pediatric recipients. We retrospective analyzed hemodynamic data of recipients with ACAD versus age and date-of-transplant matched controls. From 1982-2001, 18 cases fulfilled study entry criteria. Median age at transplant was 12 years for subjects and 8 years for controls. Median time to diagnosis of ACAD was 65 months (14.5-124 months) and 67 months (16-140 months) to arteriography for controls. The median right ventricular end-diastolic pressure (RVEDP) at diagnosis was 11.0 vs. 6.0 mmHg for controls (p = 0.003). Pulmonary capillary wedge pressure (PCWP) at diagnosis was 14.0 vs. 8.0 mmHg for controls (p = 0.001). When subdivided by severity of ACAD, the difference was greater in the moderate/severe group. Compared to the previous catheterization (median interval 10 months for subjects, 12.0 for controls ), there was an increase of 4.0 mmHg in RVEDP in ACAD subjects (n = 13, p = .003) versus 0 mmHg in controls (p = 0.042), and an increase in PCWP of 5.5 in subjects (p = .002) versus 0 mmHg in controls (p = 0.066). The presence of elevated filling pressures plus an interim increase should alert to the presence of ACAD and help guide further investigation.

The distance from the Amplatzer septal occluder to the mitral valve in patients undergoing interventional closure of defects in the oval fossa increases with growth of the patient.

The Amplatzer septal occluder is an alternative to operative closure of atrial septal defects within the oval fossa. An issue when deploying the device is its distance from the mitral valve. The purpose of this study is to determine how this distance changes with growth of the patient. We identified, through a review of charts, all patients undergoing closure of defects within the oval fossa by insertion of an Amplatzer septal occluder. Data were obtained from the echocardiogram 24 hours after closure, and at most recent follow-up, including left ventricular end-diastolic diameter, left atrial diameter, degree of mitral valvar regurgitation, body surface area, and distance from the device to the mitral valve. We divided the patients into 2 groups based upon change in body surface area. The first group had an increase in body surface area of at least 10%. All others were in the second group. We inserted 55 Amplatzer septal occluders in 54 patients. Of these we excluded 17 patients, 1 because quality of images was inadequate, 1 who underwent placement of 2 devices, 1 in whom the device embolised to the left ventricle the day after deployment, and 14 who have not yet had a follow-up echocardiogram. The group which exhibited an increase in body surface area of greater than 10% demonstrated an increase in distance from the device to the mitral valve, left ventricular end-diastolic, and left atrial diameters. Those who did not undergo significant growth had no increase in distance from the device to the mitral valve, but did have an increase in left atrial and left ventricular end-diastolic diameters. No patient developed mitral regurgitation. We conclude that, when deploying an Amplatzer septal occluder close to the mitral valve in children, the distance from the device to the mitral valve can be expected to increase with growth of the patient.

Outcomes for children with acute myocarditis.

The optimum treatment for myocarditis in children is unknown. We present outcomes for this disease as seen in a large series of children. Thus, we identified all children seen with myocarditis at Children's Hospital of Pittsburgh since 1985, including only those with biopsy-proven myocarditis, or cardiac dysfunction and proof of concomitant cardiotropic viral infection. Outcomes were defined as complete recovery, incomplete recovery, and death or transplantation. We identified 41 patients, 37 proven by histology, and 4 patients who were too unstable for biopsy but had proof of viral infection. Of the group, 27 (66%) made a complete recovery, 4 (10%) had incomplete recovery, and 10 (24%) either died (5) or underwent transplantation (5). The median time to death or transplantation was 8.4 months, with a range from 1 day to 49 months. Steroids had been administered to 16 patients, of whom 10 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. Intravenous immune globulin was given in isolation to one patient, who made a complete recovery, and to 18 in combination with steroids, of whom 12 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. The remaining 6 patients received neither steroids nor intravenous immune globulin, and of these, 4 made a complete recovery, 1 was transplanted, and 1 died. Freedom from death or transplantation was 81% at 1 year, and 74% at 5 years, with no difference between the modes of treatments. The median time to recovery of function was also comparable between the groups. Thus, in our patients, treatment with intravenous immune globulin appeared to confer no advantage to steroid therapy alone. These data emphasise the need for randomised trials to assess the efficacy of current treatments, as well as that of new therapies.

Percutaneous closure of atrial septal defect via transjugular approach with the Amplatzer septal occluder after unsuccessful attempt using the CardioSEAL device.

Percutaneous closure of a secundum atrial septal defect was performed successfully via the jugular approach in a 77-year-old patient with heparin-induced thrombocytopenia and total occlusion of the inferior vena cava using the Amplatzer septal occluder after an unsuccessful attempt using the CardioSEAL septal occluder. This case demonstrates the advantages of the jugular approach in the patient with difficult anatomy and the advantage of the Amplatzer over the CardioSEAL device in this situation.