Impact of Surgical Complexity on Health-Related Quality of Life in Congenital Heart Disease Surgical Survivors.

BACKGROUND: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors. METHODS AND RESULTS: Pediatric Cardiac Quality of Life (PCQLI) study participants who had undergone cardiac surgery were included. The PCQLI database provided sample characteristics and QOL scores. Surgical complexity was defined by the highest ABC raw score or RACHS-1 category. Relationships among surgical complexity and demographic, clinical, and QOL variables were assessed using ordinary least squares regression. A total of 1416 patient-parent pairs were included. Although higher ABC scores and RACHS-1 categories were associated with lower QOL scores (P<0.005), correlation with QOL scores was poor to fair (r=-0.10 to -0.29) for all groups. Ordinary least squares regression showed weak association with R(2)=0.06 to R(2)=0.28. After accounting for single-ventricle anatomy, number of doctor visits, and time since last hospitalization, surgical complexity scores added no additional explanation to the variance in QOL scores. CONCLUSIONS: ABC scores and RACHS-1 categories are useful tools for morbidity and mortality predictions prior to cardiac surgery and quality of care initiatives but are minimally helpful in predicting a child's or adolescent's long-term QOL scores. Further studies are warranted to determine other predictors of QOL variation.

Parachute mitral valve: morphologic descriptors, associated lesions, and outcomes after biventricular repair.

OBJECTIVE: In "true" parachute mitral valve, mitral valve chordae insert into one papillary muscle. In parachute-like asymmetric mitral valve, most or all chordal attachments are to one papillary muscle. This study compared morphologic features, associated lesions, and palliation strategies of the two parachute mitral valve and dominant papillary muscle types and examined interventions and midterm outcomes in patients with biventricular circulation. METHODS: Echocardiography and autopsy databases were reviewed to identify patients with "true" parachute mitral valve or parachute-like asymmetric mitral valve from January 1987 to January 2006. Predictors of palliation strategy in the entire cohort, mitral stenosis on initial echocardiogram, and mortality in the biventricular cohort were determined with logistic regression. RESULTS: Eighty-six patients with "true" parachute mitral valve (n = 49) or parachute-like asymmetric mitral valve (n = 37) were identified. Chordal attachments to the posteromedial papillary muscle were more common (73%). The presence "true" parachute mitral valve (P = .008), hypoplastic left ventricle (P < .001), and two or more left-sided obstructive lesions (P = .002) predicted univentricular palliation. Among 49 patients maintaining biventricular circulation at follow-up, 8 died median follow-up 6.4 years (7 days-17.8 years). Multivariate analysis revealed that "true" parachute mitral valve was associated with mitral stenosis on initial echocardiogram (P = .03), and "true" parachute mitral valve (P = .04) and conotruncal anomalies (P = .0003) were associated with mortality. Progressive mitral stenosis was found in 11 patients; 2 underwent mitral valve interventions, and 1 died. CONCLUSION: Nearly two thirds of this parachute mitral valve cohort underwent biventricular palliation. Some progression of mitral stenosis occurred, although mitral valve intervention was rare. "True" parachute mitral valve was associated with mitral stenosis on initial echocardiogram. "True" parachute mitral valve and conotruncal anomalies were associated with mortality in the biventricular population.

The development of the pediatric cardiac quality of life inventory: a quality of life measure for children and adolescents with heart disease.

OBJECTIVE: Mortality after surgery for congenital heart disease (CHD) has decreased. Quality of life (QOL) assessment in survivors has become increasingly important. The purpose of this project was to create the Pediatric Cardiac Quality of Life Inventory (PCQLI). METHODS: Items were generated through nominal groups of patients, parents, and providers. The pilot PCQLI was completed by children (age 8-12), adolescents (age 13-18), and their parents at three cardiology clinics. Item reduction was performed through analysis of items, principal components, internal consistency (IC), and patterns of correlation. RESULTS: A total of 655 patient-parent pairs completed the pilot PCQLI. Principal components identified included: impact of disease (ID); psychosocial impact (PI); and emotional environment (EE). After item reduction ID and PI had excellent IC (ID = 0.88-0.91; PI = 0.78-0.85) and correlated highly with each other (0.81-0.90) and with the total score (TS) (ID = 0.95-0.96; PI = 0.87-0.93). EE was not correlated with ID, PI, or TS and was removed from the final forms. Two-ventricle CHD patients had a higher TS than single-ventricle CHD patients across all forms (P < 0.001). CONCLUSION: The PCQLI has patient and parent-proxy forms, has wide age range, and discriminates between CHD subgroups. The ID and PI subscales of the PCQLI have excellent IC and correlate well with each other and the TS.

Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients.

OBJECTIVES: We sought to prospectively evaluate evidence of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA). BACKGROUND: An AAOCA is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. Data evaluating ischemia after AAOCA repair are limited. METHODS: We included children who underwent AAOCA surgery between October 2001 and December 2006. They were prospectively assessed with exercise stress test (EST), stress echocardiogram (SE), and stress myocardial perfusion scan (MPS). RESULTS: Of 24 participating children, 15 (63%) were male, 16 (67%) had anomalous right coronary (ARCA), and 7 (29%) were asymptomatic. Median age was 12 (5 to 18) years; follow-up was 15 (2 to 48) months. All had unobstructed neo-coronary ostia by echocardiogram and were asymptomatic. One anomalous origin of left main coronary artery (ALCA) patient and 8 ARCA patients had post-operative evaluations suggestive of ischemia. The ALCA patient had reversible apical septal and mid-anteroseptal hypokinesis on SE. Of the ARCA patients, 2 had inferior ST-segment depression on EST; subsequently, 1 had normal tests, but the other developed anterolateral Q waves. Two patients had blunted blood pressure response with EST, 1 had fixed apical inferior hypokinesis on SE, 2 had reversible perfusion defects on MPS, and 1 had a fixed perfusion defect on MPS. CONCLUSIONS: Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair. The implication of these results on indication for surgery and subsequent sudden death risk is unknown. Serial EST, SE, and MPS are essential in evaluating ongoing ischemia risk after AAOCA repair.