Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis.

OBJECTIVES: Evaluate the incidence of various morphologic types of congenital AS, and the association between valve morphology and long-term outcomes, including repeat BAV, AVR, and death/transplant. BACKGROUND: Reports on long-term outcomes have low agreement on the influence of morphologic type. METHODS: We queried our institutional database and hospital billing records to identify all patients who underwent balloon aortic valvuloplasty (BAV) from 1992 through 2009. We excluded cases where morphology was not clear based on the description in the pre-BAV echocardiogram report and patients who underwent single ventricle palliation. The primary outcome of the study was the occurrence of any of the following events: repeat valvuloplasty, AVR, heart transplant, or death. RESULTS: There were 147 patients in our study cohort. The most common morphology was functionally bicuspid (n = 92, 63%), followed by functionally unicuspid (n = 20, 14%), dysplastic (n = 16, 11%), true bicuspid (n = 13, 9%), and true unicuspid (n = 6, 4%). The primary endpoint was less likely to occur in patients with functionally bicuspid valves (P < 0.01) and patients with true bicuspid valves (P = 0.03), whereas it was more likely to occur in patients with functionally unicuspid valves (P = 0.02) and patients with true unicuspid valves (P = 0.05). Multivariate Cox regression analysis demonstrated that valve type other than functionally bicuspid was associated with diminished freedom from repeat intervention, death or transplant (HR 3.3, CI 1.2 - 8.6, P = 0.02). CONCLUSIONS: In our cohort, patients with functionally bicuspid aortic valves, the most common type, had improved outcomes as compared with all other morphologic types.

Repeat balloon aortic valvuloplasty effectively delays surgical intervention in children with recurrent aortic stenosis.

OBJECTIVE: Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Recurrent AS following initial BAV or initial surgical valvotomy (SV) may require a second BAV (BAV2). We sought to determine the longterm outcomes of BAV2. DESIGN: We reviewed all cases of BAV2, defined as BAV following primary BAV or SV between 1988 and 2009. Cases were reviewed for pre- and post-BAV2 echocardiographic and procedural details. SETTING: Tertiary care dedicated children's hospital. PATIENTS: Between 1985 and 2009, 43 patients underwent BAV2 (23 primary SV, 20 primary BAV) at median age 1.9 years (1 month-21 years) and median weight 15 (3.3-55) kg. INTERVENTIONS: BAV2 performed following primary SV or primary BAV. MAIN OUTCOME MEASURES: We evaluated the following endpoints: ≥ moderate AI post-BAV2, aortic valve replacement (AVR), additional BAV or SV post-BAV2, death and heart transplantation. RESULTS: The gradient decreased from 61.4 ± 16.0 mm Hg to 26.0 ± 13.6 post-BAV2 (P < 0.01). Gradient prior to BAV2 was higher in primary SV patients (66 ± 13 mm Hg) than in primary BAV patients (56 ± 18 mm Hg, P = 0.04). 24 patients had no further events after BAV2, while 19 patients (44%) experienced 23 events including: AVR (n = 8), SV (n = 6), BAV3 (n = 2), death (n = 5), and transplant (n = 1). Regression demonstrated that adverse events were associated with higher post-BAV2 gradient (P < 0.01). Repeat intervention on the aortic valve and AVR were associated with higher post BAV2 gradient (P = 0.04, P = 0.01). Prior to BAV2, 7 patients (17%) had AI > mild, compared to 21 (51%) patients after BAV2. Cox regression revealed that primary BAV was associated with development of AI > mild after BAV2 (P < 0.01). CONCLUSION: BAV2 is associated with decreased valve gradient, though with an increase in AI. However, residual AS, not AI, is associated with poor outcomes following BAV2. BAV2 effectively treats recurrent AS and postpones need for surgical intervention.

Diminished left ventricular function is associated with poor mid-term outcomes in neonates after balloon aortic valvuloplasty.

OBJECTIVES: We studied outcomes of neonatal aortic valvuloplasty to determine the risk factors for poor outcomes. BACKGROUND: Balloon aortic valvuloplasty (BAV) is the primary therapy for neonates with severe aortic stenosis (AS). Limited data are available on the mid-term and long-term outcomes in this population, and reported risk factors for poor outcomes vary among studies. METHODS: We reviewed all cases of BAV in neonates in our institution between 1998 and 2009. We reviewed patient characteristics, preintervention echocardiographic, and procedural details. We tracked repeat BAV, aortic valve replacement (AVR), and death/transplant. Kaplan-Meier and Cox regression analyses were performed. Changes in dimensions of left heart structures post-BAV were also studied. RESULTS: Forty-eight neonates were included-of these, 30 (62%) had critical AS. There was one procedural death. The remaining 47 neonates were followed for 4.8 ± 4.4 years. Repeat BAV was performed in 19 (40%) neonates; AVR was performed in 9 (19%), and death/transplant occurred in 4 (8%) neonates. Multivariate analysis revealed that lower LV shortening fraction pre-BAV, higher pre-BAV and final valve gradient, and need for inotropes were associated with poor long-term outcomes. LV diastolic and systolic dimensions normalized over time, and left heart structures improved in size early and late after BAV. CONCLUSIONS: Neonatal BAV is associated with low mortality. Lower LV shortening fraction pre-BAV is associated with risk of future interventions, while repeat valvuloplasty is safe and effective for recurrent AS. There is significant improvement in dimensions of the LV and aortic valve annulus following BAV.

Twenty-five year experience with balloon aortic valvuloplasty for congenital aortic stenosis.

Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Few reports describe long-term outcomes. In this study, a retrospective single-institution review was performed of patients who underwent BAV for congenital AS. The following end points were evaluated: moderate or severe aortic insufficiency (AI) by echocardiography, aortic valve replacement, repeat BAV, surgical aortic valvotomy, and transplantation or death. From 1985 to 2009, 272 patients who underwent BAV at ages 1 day to 30.5 years were followed for 5.8 ± 6.7 years. Transplantation or death occurred in 24 patients (9%) and was associated with depressed baseline left ventricular shortening fraction (LVSF) (p = 0.04). Aortic valve replacement occurred in 42 patients (15%) at a median of 3.5 years (interquartile range 75 days to 5.9 years) after BAV and was associated with post-BAV gradient ≥25 mm Hg (p = 0.02), the presence of post-BAV AI (p = 0.03), and below-average baseline LVSF (p = 0.04). AI was found in 83 patients (31%) at a median of 4.8 years (interquartile range 1.4 to 8.7) and was inversely related to post-BAV gradient ≥25 mm Hg (p <0.04). AI was associated with depressed baseline LVSF (p = 0.02). Repeat valvuloplasty (balloon or surgical) occurred in 37 patients (15%) at a median of 0.51 years (interquartile range 0.10 to 5.15) and was associated with neonatal BAV (p <0.01), post-BAV gradient ≥25 mm Hg (p = 0.03), and depressed baseline LVSF (p = 0.05). In conclusion, BAV confers long-term benefits to most patients with congenital AS. Neonates, patients with post-BAV gradients ≥25 mm Hg, and patients with lower baseline LVSF experienced worse outcomes.

Impact of pharmacotherapy on interstage outcomes in single ventricle infants.

OBJECTIVE: To characterize the pharmacotherapeutic regimens used in infants with single ventricle heart disease and determine the influence of outpatient medications on interstage weight gain. DESIGN: Retrospective review. SETTING: Tertiary care pediatric hospital. PATIENTS: All patients discharged from our institution with single ventricle heart disease that underwent neonatal first stage surgical palliation between 2002 and 2009 were included. Patients who died prior to second stage palliation or underwent orthotopic heart transplantation were excluded. OUTCOME MEASURES: Outpatient medication regimens during the interstage period were reviewed. Medication regimens were compared between surgical eras and between patient groups experiencing different outcomes. A logistic regression model was developed to determine independent factors for an interstage increase in weight-for-age z-score (WAZ) and a linear regression model to determine medications significant for an increase in weight gain per day. RESULTS: The study cohort consisted of 161 patients (58% male). Most patients in this cohort had either hypoplastic left heart syndrome (51%) or unbalanced complete atrioventricular canal (29%). Patients were placed on a median of four medications (range 1-9) at discharge from first surgical palliation, with aspirin (79%), furosemide (79%), and angiotensin converting enzyme inhibitors (ACE-I) (73%) most commonly prescribed. A median of six medication doses per day (range 2-18) were prescribed at discharge. Most patients (71%) had a decrease in WAZ during the interstage period. Use of digoxin (P < 0.01) and high-dose furosemide (P = .02) were associated with a decrease in WAZ score during the interstage period. Additionally, the use of ACE-I, ranitidine, proton-pump inhibitors, or promotility agents was not associated with improved somatic growth during the interstage period. CONCLUSIONS: Infants with single ventricle heart disease have a high-medication burden during the interstage period. Despite the focused and intensified use of medications to improve feeding tolerance and somatic growth, current pharmacotherapeutic regimens appear to have little effect on interstage weight gain.

The impact of a dedicated single-ventricle home-monitoring program on interstage somatic growth, interstage attrition, and 1-year survival.

OBJECTIVE: There has been considerable improvement in survival after the first stage of palliation for single-ventricle heart disease. Yet, interstage mortality continues to plague this population. Home monitoring has been proposed to reduce interstage mortality. We review our experience after creation of a Single Ventricle Program. METHODS: All infants with a single ventricle heart defect who were admitted to Texas Children's Hospital from the inception of the Single Ventricle Program on September 1, 2007, to January 1, 2010, were included in the Single Ventricle Program cohort. Infants with a single ventricle presenting between January 1, 2002, and August 31, 2007, comprised the pre-Single Ventricle Program group. Anatomic, operative, and postoperative details were noted for all patients. End points included in-hospital death after the first stage of palliation, interstage death (defined as after discharge from the first stage of palliation and before the second stage of palliation), and death or heart transplantation by 1 year of age. Interstage weight gain was also compared. RESULTS: A total of 137 infants with a single ventricle were included in the pre-Single Ventricle Program cohort, and 93 infants were included in the Single Ventricle Program cohort. Anatomic subtypes were similar between groups. There was significant improvement in rate of interstage weight gain, whereas age at the second stage of palliation was significantly reduced in the Single Ventricle Program group. In-house mortality decreased during the Single Ventricle Program era (P = .021). Interstage mortality did not significantly decrease in the Single Ventricle Program group. However, 1-year transplant-free survival improved during the Single Ventricle Program era (P = .002). CONCLUSIONS: The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program.

Outcomes of transcatheter occlusion of patent ductus arteriosus in infants weighing ≤ 6 kg.

OBJECTIVES: We sought to analyze the outcomes of transcatheter patent ductus arteriosus (PDA) occlusion using a variety of devices in infants weighing ≤6 kg. BACKGROUND: Indications for transcatheter closure of a PDA in infancy include congestive heart failure and/or failure to thrive. Devices available for small infants may be problematic for various reasons, including sheath size, stiffness of delivery system, and anchoring and retrievability characteristics of the device. The Amplatzer Ductal Occluder is approved by U.S. Food and Drug Administration for children weighing >6 kg and older than 6 months of age. METHODS: We performed a multicenter, retrospective analysis of children weighing ≤6 kg in whom transcatheter PDA occlusion was attempted between January 1995 and November 2005 at Texas Children's Hospital and January 2001 to November 2005 at Children's Hospital of San Diego. RESULTS: A total of 62 patients underwent attempted closure. The mean age at catheterization was 4.7 ± 2.8 months with a mean weight at catheterization of 4.6 ± 0.9 kg. Successful device placement was achieved in 58 of 62 patients (94%). Among those receiving a device, complete occlusion was noted in all 58 patients at either catheterization or last available follow-up. CONCLUSIONS: Percutaneous closure of PDA should be considered even in infants ≤6 kg.