Health-related quality of life outcomes in children and adolescents with congenital heart disease.

OBJECTIVES: To compare health-related quality of life (HRQOL) in a group of pediatric patients with congenital heart disease (CHD) and healthy controls and patients with other chronic diseases, and to compare HRQOL among patients with CHD of various severity categories with one another, with controls, and with patients with other chronic diseases. STUDY DESIGN: In this cross-sectional survey, t tests were used to compare patient and proxy-reported Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL) scores (including total, physical health, and psychosocial health summary scores) in children (aged 8-12 years) and adolescents (aged 13-18 years) between controls and (1) a composite CHD population; and (2) patients in each of 3 CHD severity categories: mild (no intervention), biventricle (BV; postintervention), and single ventricle (SV; postpalliation). PedsQL scores among CHD severity categories were compared by ANOVA. PedsQL scores were also compared in the CHD population and children with other chronic diseases without age stratification using t tests. RESULTS: There were 1138 (children, n = 625; adolescents, n = 513) and 771 (children, n = 528; adolescents, n = 243) patient and/or proxy reporters in the CHD and healthy control groups, respectively. Total, physical health, and psychosocial health summary scores were lower in the composite CHD, BV, and SV groups compared with controls (P < .0001). There were significant differences among disease severity categories for all scores (P < .01). The composite CHD, BV, and SV groups had similar PedsQL scores as end-stage renal disease, asthma, and obesity populations. CONCLUSION: Children and adolescents with BV and SV CHD have significantly lower HRQOL than healthy controls and similar HRQOL as patients with other chronic pediatric diseases. Interventions targeting both physical and psychosocial domains are needed to improve HRQOL in this high-risk population.

Correlation of echocardiographic markers and therapy in persistent pulmonary hypertension of the newborn.

BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) causes morbidity and mortality in neonates. High-frequency ventilation (HFV), inhaled nitric oxide (iNO), and extracorporeal membrane oxygenation (ECMO) are used when conventional treatment fails. This study aimed to identify echocardiographic predictors of progression to these therapies before clinical deterioration. METHODS: Echocardiographic parameters were compared for survival and need for ECMO, HFV, iNO, and prolonged mechanical ventilation (MV, >or=10 days). RESULTS: Of 63 neonates, 95% survived, with 14% requiring ECMO, 52% requiring HFV, 67% requiring iNO, and 35% requiring MV. The following echocardiographic indices reflecting left ventricular output were decreased in sicker infants: (1) A decreased ascending aortic velocity time integral indicated an increased likelihood of ECMO (p=0.02), iNO (p=0.01), or MV (p=0.05), (2) Shorter transverse aortic arch antegrade ejection time indicated HFV (p<0.01), iNO (p<0.01), and MV (p=0.03), (3) Absent or retrograde transverse aortic diastolic flow correlated with HFV (p=0.01, iNO (p=0.01), and MV (p<0.01). These sicker patients were more likely to have smaller left ventricular end-diastolic areas (p<0.03 for all) and right-to-left atrial shunting (ECMO, HFV, and MV). There were no differences in survival. CONCLUSIONS: Decreased left ventricular size and output correlates with the need for advanced therapies in infants with PPHN. Early identification may allow more effective management and placement of neonates at risk.

Digital imaging, archiving, and structured reporting in pediatric echocardiography: impact on laboratory efficiency and physician communication.

BACKGROUND: Digital structured reporting (DSR) is an emerging technology in medical information management. In November of 2006, the pediatric echocardiography laboratory at Children's Hospital of Wisconsin transitioned from transcription-based reporting of results to DSR. METHODS: Transthoracic echocardiograms were reviewed for the study duration and time to report completion for October of 2006 (the last month of phone-based transcription) and January of 2007 (the third month of DSR). For both months, the 5 cardiologists responsible for echocardiography reporting were evaluated for report generation time and the 5 sonographers were evaluated for study duration. Data for physicians and sonographers were analyzed individually, as a group between months, between examination types (brief vs comprehensive), and diagnoses (normal vs abnormal findings). RESULTS: The time to generate a final report to the electronic medical record/auto-fax to the referring physician for all examination types between months decreased significantly from the transcription month to the DSR month (median 23.8 vs 1.2 hours; P =.001). This decrease was consistent among physicians and occurred despite an increase in monthly echocardiography volume of 12% between the study periods. No difference was noted in report generation times between brief and comprehensive transthoracic echocardiography for transcription (P =.220) or DSR (P =.185). Analysis of diagnoses after instituting DSR revealed significantly shorter report generation time for normal examination findings versus abnormal examination findings (0.97 vs 1.5 hours; P =.001). Study duration decreased significantly from the transcription month to the DSR month for all examination types (32 vs 27 minutes; P =.001). For both months, the study duration was longer for abnormal versus normal transthoracic echocardiography findings (transcription: 37 vs 27 minutes; DSR: 32 vs 23 minutes). CONCLUSIONS: DSR is a superior technology for pediatric echocardiography reporting and an important next step to increase efficiency and customer satisfaction. Although congenital heart disease increases DSR study report time, the value of digitally scripted, database-compatible, complete segmental analysis of the heart and vascular structures with direct transfer of quantitative data from the ultrasound machine to the report without the need for a transcription interface justifies the time expense. DSR should be a goal for all pediatric echocardiography laboratories to decrease consumer wait time, increase laboratory efficiency, and improve compliance with Intersocietal Commission for the Accreditation of Echocardiography Laboratories standards.

Effect of shunt type on echocardiographic indices after initial palliations for hypoplastic left heart syndrome: Blalock-Taussig shunt versus right ventricle-pulmonary artery conduit.

OBJECTIVES: The study objective was to compare echocardiographic indices in infants with hypoplastic left heart syndrome (HLHS) randomized to different surgical pathways. BACKGROUND: Initial surgical palliation for HLHS has evolved to two strategies that vary by source of pulmonary blood flow: a modified Blalock-Taussig (BT) shunt or a right ventricle to pulmonary artery (RV-PA) conduit. METHODS: Seventeen patients were randomized to either a BT shunt or RV-PA as their first-stage palliation, and 15 survived through bidirectional Glenn (BDG) (seven BT shunts/eight RV-PAs). Echocardiography was performed pre-stage 1 palliation (S1P), early post-S1P, pre-BDG, and post-BDG. Echocardiographic measurements included indices of right ventricle function and shape, right ventricle myocardial performance index, neoaortic Doppler flow patterns and cardiac output, Doppler inflow and tissue imaging, and conduit/neoaortic regurgitant fraction. RESULTS: There were no significant differences in right ventricle size, shape, or estimates of systolic and diastolic function between groups. Patterns of changes in neoaortic Doppler flow in the RV-PA conduit group showed a decrease in all indices of systemic flow early after initial palliation, but these measures consistently increase later post-S1P and post-BDG. In contrast, the BT shunt group increases these same indices post-S1P and pre-BDG with a decrease after shunt removal. CONCLUSIONS: Right ventricle size and function do not appear significantly affected by surgical type of initial palliation for HLHS, and this correlates with early outcome. Echocardiographic Doppler findings correlate well with the expected physiologies of the different shunts as these infants progress through initial palliative strategies.

Early cavopulmonary anastomosis after Norwood procedure results in excellent Fontan outcome.

BACKGROUND: Children with univentricular hearts and aortic arch obstruction are treated sequentially with Norwood procedure, superior cavopulmonary anastomosis (SCPA), and Fontan operation. Early SCPA results in lower initial O2 saturation and longer hospitalization, but not increased mortality. We sought to determine the impact of early SCPA on Fontan candidacy and outcomes. METHODS: Eighty-five consecutive patients undergoing Norwood operation between January 1998 and February 2003 were divided into group 1 (SCPA at less than 4 months, n = 33) and group 2 (SCPA at more than 4 months, n = 52). Of the original cohort, 69 have undergone Fontan operation, 7 await Fontan, 1 was transplanted, 3 are not Fontan candidates, and 5 died late after SCPA. Group 1 (n = 25) and group 2 (n = 44) patients who have completed Fontan operation were compared for preoperative and perioperative variables: age, size, O2 saturation, pulmonary artery pressure and size, prevalence of tricuspid regurgitation and ventricular dysfunction, extubation rate in operating room, duration of pleural drainage, hospital stay, and discharge O2 saturation. Late functional status and ventricular function were also compared. Survival was compared for original groups 1 and 2. RESULTS: There were no differences for any preoperative or perioperative variable, or late functional assessment. Actuarial survival at 6 years was also not different (88% +/- 5% for group 1 and 94% +/- 4% for group 2, p = 0.72). CONCLUSIONS: Although initially more cyanotic and hospitalized longer than older peers, younger SCPA patients achieve clinical equivalence by the time of Fontan operation and afterward. We conclude that both short- and long-term outcomes support performance of early SCPA.

Presentation and echocardiographic markers of neonatal hypertensive cardiomyopathy.

BACKGROUND: Systemic hypertension is a rare but important cause of neonatal heart failure. It is critical that this etiology be recognized and distinguished from other causes of myocardial dysfunction in young infants, because diagnostic studies, treatments, and prognoses are quite different. METHODS: Between 1991 and 2005, 11 neonates were diagnosed as having neonatal cardiomyopathy and systemic hypertension through retrospective review of the Children's Hospital of Wisconsin database. RESULTS: All infants in the cohort were found to have systemic hypertension (blood pressure of > 95th percentile for gestational age and weight). Causes included renovascular disease (n = 9), aortic obstruction secondary to thrombus (n = 1), and steroid use (n = 1). Echocardiography was performed at presentation for all patients because of cardiomegaly and/or hypertension. Echocardiographic findings were consistently striking for (1) left ventricular systolic dysfunction without chamber dilation, (2) concentric left ventricular hypertrophy, (3) left atrial dilation, and (4) aortomegaly. No anatomic aortic arch obstruction was identified, but Doppler findings for the descending thoracic aorta were uniformly consistent with elevated systemic vascular resistance. One patient died as a result of overwhelming thrombotic disease; all other patients responded to afterload reduction therapy with normalization of left ventricular systolic function during infancy. CONCLUSIONS: Hypertensive cardiomyopathy can present in neonates with nonspecific symptoms and systemic hypertension. Because sometimes hypertension in infants is ignored or misinterpreted as agitation, echocardiography can provide critical markers of the disease.

Long-term and developmental outcomes of children with complex congenital heart disease.

As short-term survival of complex congenital heart disease continues to improve dramatically with advances in medical and surgical treatment, further efforts must be made to understand the long-term outcomes of our efforts. As survival continues to improve, cardiovascular morbidity and, equally importantly, neurodevelopmental and social outcomes must be a continual focus in our treatment of these complex patients. Further study of these effects is underway, and more is certainly warranted. Understanding should lead to modification of current techniques and management strategies, all with the ultimate goal of improving our patients' quality of life.

Alteration of the critical arteriovenous oxygen saturation relationship by sustained afterload reduction after the Norwood procedure.

OBJECTIVES: Hemodynamic vulnerability after the Norwood procedure for hypoplastic left heart syndrome results from impaired myocardial function, and critical inefficiency of parallel circulation. Traditional management strategies have attempted to optimize circulatory efficiency by using arterial oxygen saturation (SaO(2)) as an index of pulmonary/systemic flow balance, attempting to maintain SaO(2) within a theoretically optimal critical range of 75% to 80%. This optimal range of SaO(2) has not been verified clinically, and strategies targeting SaO(2) may be limited by the fact that SaO(2) is a poor predictor of systemic oxygen delivery. We have previously reported higher venous saturation (SvO(2)), lower arteriovenous oxygen content difference, lower systemic vascular resistance, lower pulmonary/systemic flow ratio, and improved survival with the perioperative use of phenoxybenzamine and continuous monitoring of SvO(2). In this investigation, we tested the hypothesis that intense afterload reduction with phenoxybenzamine would modify the SvO(2)-SaO(2) relationship by preventing deterioration of systemic oxygen delivery at high SaO(2). METHODS: Seventy-one consecutive neonates undergoing the Norwood procedure with and without phenoxybenzamine were studied. Perioperative hemodynamic management targeted SvO(2) greater than 50%. Hemodynamic data were prospectively acquired for 48 hours postoperatively and analyzed to assess the effect of phenoxybenzamine on the relationship between SaO(2) and SvO(2) and other hemodynamic indices. Sixty-two patients received phenoxybenzamine 0.25 mg/kg on cardiopulmonary bypass; 9 who did not served as controls. RESULTS: In control patients, SvO(2) peaked at an SaO(2) of 77%, with reduced SvO(2) at SaO(2) > 85% and SaO(2) < 70% (P <.01), while arteriovenous oxygen content difference increased with SaO(2) greater than 80% (P <.001). In patients receiving phenoxybenzamine, the SvO(2) increased linearly with SaO(2) greater than 65% (P <.001), and arteriovenous oxygen content difference was constant at all SaO(2) (P = ns). The SvO(2) was higher, and the arteriovenous oxygen content difference lower, across the whole SaO(2) range with phenoxybenzamine (P <.0001). CONCLUSIONS: A critical range of SaO(2) for optimizing systemic oxygen delivery was confirmed in control patients, and was effectively eliminated by phenoxybenzamine, specifically by eliminating the systemic hypoperfusion associated with high SaO(2). This effect allows higher SaO(2) to be included in a rational hemodynamic strategy to improve systemic oxygen delivery in the early postoperative management of patients receiving intense afterload reduction with phenoxybenzamine. The predictability of SvO(2) from SaO(2) is low in both groups, emphasizing the importance of SvO(2) measurement in these patients.