Left ventricular function by pressure-volume loop analysis before and after percutaneous repair of large atrial septal defects.

AIM: The intent of the present study was to evaluate changes in ventricular function with percutaneous closure of atrial septal defect (ASD), as it is associated with alterations in ventricular loading and function. Transcatheter occlusion of ASD imparts acute changes in volume loading of the left ventricle (LV) that obscures measurement of ventricular function by load-dependent indices. To differentiate between changes in ventricular loading and function, load-independent indices of ventricular function must be utilized. METHODS: During transcatheter occlusion of ASD, subjects underwent measurement of LV pressure and volume by the conductance catheter method. Load-dependent indices of ventricular function included: systolic and diastolic pressures, +dP/dtmax , and -dP/dtmax . Load-independent indices included: elastance and tau, the preload-independent time constant ofisovolumic relaxation. To obtain elastance, afterload was augmented by phenylephrine bolus pre- and post-device occlusion. RESULTS: In total, 29 patients (age 2-79 years) underwent ASD device occlusion (device size 12-38 mm, median 28 mm). Load-dependent indices were obtained in all, and satisfactory pressure-volume loops in 11. At baseline, LV end-diastolic pressure was 5-23 mmHg (13 ± 5 mmHg) and tau was 31 ± 6 ms. Postclosure of the ASD, LV systolic and diastolic pressures rose by 10 ± 11 mmHg and 5 ± 3 mmHg, respectively (P < 0.05), and +dP/dtmax rose from 1,288 ± 313 mmHg/sec to 1,415 ± 465 mmHg/sec (P < 0.05), but -dP/dtmax was unchanged. Elastance significantly improved (9.4 ± 8.3 mmHg/mL vs. 13.0 ± 7.3 mmHg/mL, P < 0.05) and tau was unchanged. CONCLUSIONS: Transcatheter occlusion of ASD is associated with acute improvement in load-independent indices of systolic function in this cohort, without significant worsening of the preload-independent index of diastolic function.

Effects of race, ethnicity, and gender on surgical mortality in hypoplastic left heart syndrome.

Information is limited regarding the effect of race, ethnicity, and gender on the outcomes of the three palliative procedures for hypoplastic left heart syndrome (HLHS). This study examined the effects of race, ethnicity, gender, type of admission, and surgical volume on in-hospital mortality associated with palliative procedures for HLHS between 1998 and 2007 using data from the University HealthSystem Consortium. According to the data, 1,949 patients underwent stage 1 palliation (S1P) with a mortality rate of 29 %, 1,279 patients underwent stage 2 palliations (S2P) with a mortality rate of 5.4 %, and 1,084 patients underwent stage 3 palliation (S3P) with a mortality rate of 4.1 %. The risk factors for increased mortality with S1P were black and "other" race, smaller surgical volume, and early surgical era. The only risk factors for increased mortality with S2P were black race (11 % mortality; odds ratio [OR], 3.19; 95 % confidence interval [CI] 1.69-6.02) and Hispanic ethnicity (11 % mortality; OR 3.30; 95 % CI 1.64-6.64). For S2P, no racial differences were seen in the top five surgical volume institutions, but racial differences were seen in the non-top-five surgical volume institutions. Mortality with S1P was significantly higher for patients discharged after birth (37 vs 24 %; p = 0.004), and blacks were more likely to be discharged after birth (12 vs 5 % for all other races; p < 0.001). No racial differences with S3P were observed. The risk factors for increased mortality at S1P were black and "other" race, smaller surgical volume, and early surgical era. The risk factors for increased in-hospital mortality with S2P were black race and Hispanic ethnicity.

Frequency and degree of change of peak transvalvular pressure gradient determined by two Doppler echocardiographic examinations in newborns and children with valvular congenital aortic stenosis.

The natural history of congenital aortic stenosis (AS) has been described predominantly using data derived from catheterization studies. Because of the inherent selection bias of many of these studies, this study was conducted to determine the course of valvular AS using data from serial echocardiograms, for which this bias is less pervasive. The medical records of 103 patients (mean age 3.3+/-3.8 years at time of diagnosis) with congenital AS were examined, and data from the initial and most recent echocardiograms before any intervention on the aortic valve were recorded. The average change in gradient for the entire study population was +1 mm Hg/patient-year. Forty percent (8 of 20) of patients diagnosed in the newborn period (<2 months) required intervention before 6 months of age, compared with 11% (9 of 83) of those diagnosed after the newborn period who required intervention at any time during the study period (p<0.01). Patients diagnosed at >or=1 year of age were less likely to have acute increases in gradient or need intervention; those older patients whose gradients progressed more rapidly were more likely to have significant (more than mild) aortic insufficiency than those whose gradients did not progress (55% vs 28%, p<0.05). In conclusion, congenital AS is usually a slowly progressive disease. Risk factors for more rapid gradient progression include diagnosis as a neonate and the development of significant aortic insufficiency.

Management of hypoplastic left heart syndrome in the 1990s.

We used the discharge database of the University Hospital Consortium to determine the management and early outcome of neonates with hypoplastic left heart syndrome admitted to member institutions from 1990 to 1999. Of the 2,264 patients, 1,203 underwent a Norwood procedure, with 42% mortality. Cardiac transplantation was performed in 72, with 38% mortality, and 217 (10%) were discharged without any surgical procedure. The proportion of patients managed by the Norwood procedure increased from 43% during the first half of the decade to 59% in the second half, with corresponding decreases in the proportion managed by transplantation or nonintervention. A mortality rate of < or =40% was achieved in all 5 institutions performing >50 Norwood procedures, and by 9 of 40 institutions performing <50. Performance of a Norwood procedure has become the most frequent management for neonates with hypoplastic left heart syndrome. Lower operative mortality rates are generally, but not exclusively, achieved by institutions with high surgical volume.

Pediatric circulatory support systems.

Ventricular assist devices (VADs) are a valid option for long term circulatory support in pediatric patients with postoperative myocardial failure or debilitating heart defects. Most clinical experience to date has involved the short-term support of patients weighing 6 kg and larger. For cases of VAD implementation in pediatric patients, the assist device showed tremendous promise in reversing cardiac failure and providing adequate support as a bridge to cardiac transplantation. The Medos-HIA system, Berlin Heart, Medtronic Bio-Medicus Pump, Abiomed BVS 5000, Toyobo-Zeon pumps, and Hemopumps have proven successful for short-term circulatory support for the pediatric population. The Jarvik 2000 and Pierce-Donachy pediatric system further demonstrate the potential to be used for pediatric circulatory support. The clinical and experimental success of these support systems provide encouragement to believe that long-term support is possible.

Predicting economic and medical outcomes based on risk adjustment for congenital heart surgery classification of pediatric cardiovascular surgical admissions.

The Risk Adjustment for Congenital Heart Surgery (RACHS-1) classification is an established method for predicting mortality for congenital heart disease surgery. It is unknown if this extends to the cost of hospitalization or if differences in economic and medical outcomes exist in certain subpopulations. Using data obtained from the University HealthSystem Consortium, we examined inpatient resource use by patients with International Classification of Diseases, Ninth Revision, procedure codes representative of RACHS-1 classifications 1 through 5 and 6 from 2006 to 2012. A total of 15,453 pediatric congenital heart disease surgical admissions were analyzed, with overall mortality of 4.5% (n = 689). As RACHS-1 classification increased, the total cost of hospitalization, hospital charges, total length of stay, length of intensive care unit stay, and mortality increased. Even when controlled for RACHS-1 classification, black patients (n = 2034) had higher total costs ($96,884 ± $3,392, p = 0.003), higher charges ($318,313 ± $12,018, p <0.001), and longer length of stay (20.4 ± 0.7 days, p <0.001) compared with white patients ($85,396 ± $1,382, $285,622 ± $5,090, and 18.0 ± 0.3 days, respectively). Hispanic patients had similarly disparate outcomes ($104,292 ± $2,759, $351,371 ± $10,627, and 23.0 ± 0.6 days, respectively) and also spent longer in the intensive care unit (14.9 ± 0.5 days, p <0.001). In conclusion, medical and economic measures increased predictably with increased procedure risk, and admissions for black and Hispanic patients were longer and more expensive than those of their white counterparts but without increased mortality.

Screening for cardiovascular disease in the young.

America's youth have been screened for cardiovascular disorders for nearly a century. Initial efforts to detect rheumatic heart disease in schoolchildren have gradually shifted to the detection of congenital heart disease in the fetus and newborn, and to the prevention of sudden cardiac death in older children, especially athletes. Proposed screening programs need to consider sensitivity and specificity, cost, resource availability, and the potential for adverse effects of the screening process.

Inpatient costs and charges for surgical treatment of hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome (HLHS) is one of the most serious congenital cardiac anomalies. Typically, it is managed with a series of 3 palliative operations or cardiac transplantation. Our goal was to quantify the inpatient resource burden of HLHS across multiple academic medical centers. METHODS: The University HealthSystem Consortium is an alliance of 101 academic medical centers and 178 affiliated hospitals that share diagnostic, procedural, and financial data on all discharges. We examined inpatient resource use by patients with HLHS who underwent a staged palliative procedure or cardiac transplantation between 1998 and 2007. RESULTS: Among 1941 neonates, stage 1 palliation (Norwood or Sano procedure) had a median length of stay (LOS) of 25 days and charges of $214,680. Stage 2 and stage 3 palliation (Glenn and Fontan procedures, respectively) had median LOS and charges of 8 days and $82,174 and 11 days and $79,549, respectively. Primary neonatal transplantation had an LOS of 87 days and charges of $582,920, and rescue transplantation required 36 days and $411,121. The median inpatient wait time for primary and rescue transplants was 42 and 6 days, respectively. Between 1998 and 2007, the LOS for stage 1 palliation increased from 16 to 28 days and inflation-adjusted charges increased from $122,309 to $280,909, largely because of increasing survival rates (57% in 1998 and 83% in 2007). CONCLUSIONS: Patients with HLHS demand considerable inpatient resources, whether treated with the Norwood-Glenn-Fontan procedure pathway or cardiac transplantation. Improved survival rates have led to increased hospital stays and costs.

Use of an administrative database to determine clinical management and outcomes in congenital heart disease.

We review our 16-year experience using the large, multi-institutional database of the University HealthSystem Consortium to study management and outcomes in congenital heart surgery for hypoplastic left heart syndrome, transposition of the great arteries, and neonatal coarctation. The advantages, limitations, and use of administrative databases by others to study congenital heart surgery are reviewed.

Three-stage palliation of hypoplastic left heart syndrome in the University HealthSystem Consortium.

OBJECTIVE: The objective of this study was to describe current management and short-term outcomes for patients with hypoplastic left heart syndrome representative of nationwide experience. Additionally, this study identifies risk factors associated with mortality in patients undergoing staged surgical palliation. DESIGN: The University HealthSystem Consortium database was queried to identify all hospital admissions between 1998 and 2007 with a diagnosis of hypoplastic left heart syndrome. Procedure codes were used to determine surgical management, and outcomes were ascertained by discharge status (discharged, transferred, or expired). RESULTS: Discharge data were present from 118 hospitals in the United States. First-stage surgical palliation was performed in 1949 neonates with 30% mortality. Mortality decreased from 43% in 1998 to 18% in 2007. Large institutional case volume and later era of surgery were associated with improved operative mortality in first-stage palliation. Primary cardiac transplantation was performed in 28 neonates, and rescue transplantation in 11, with 36% mortality. Second-stage palliation was performed in 1244 patients with 5.2% mortality, and third-stage palliation was performed in 1084 patients with 4.1% mortality. An additional 62 patients over 1 month of age at time of admission received cardiac transplantation with 15% mortality. CONCLUSIONS: First-stage palliative mortality for hypoplastic left heart syndrome fell dramatically over the past decade, while that for second- and third-stage procedures remained stable. The cumulative operative mortality for three-staged repair of hypoplastic left heart syndrome was 39% over the decade, but fell to 24% for procedures in 2007.

Hybrid palliation in hypoplastic left heart syndrome.

PURPOSE OF REVIEW: Despite progressive improvement in surgical results, hypoplastic left heart syndrome remains one of the congenital heart abnormalities with the greatest morbidity and mortality. Hybrid approaches to management, combining surgical and interventional catheterization procedures, have been introduced to minimize exposure to cardiopulmonary bypass, and improve outcomes for these high-risk infants. RECENT FINDINGS: First-stage palliation of hypoplastic left heart syndrome has been performed as a hybrid procedure combining surgical pulmonary artery banding with catheterization stenting of the ductus arteriosus and balloon atrial septostomy, especially in high-risk patients. Additionally, several centers have performed second-stage palliation - bidirectional Glenn or hemi-Fontan procedures - in a manner that allows the subsequent 'Fontan' procedure to be completed in the catheterization laboratory with a covered stent. SUMMARY: These innovative procedures offer the potential of an alternative management strategy for hypoplastic left heart syndrome. They have been applied to a very limited number of patients and long-term results are not available. Their role in management of hypoplastic left heart syndrome remains to be defined, especially as results of conventional surgical management continue to improve.

Transesophageal echocardiographic guidance for surgical repair of aortic insufficiency in congenital heart disease.

A total of 14 patients with congenital heart disease underwent aortic valve repair, with transesophageal echocardiograms performed to determine severity, mechanism, and direction of aortic insufficiency (AI) jet to tailor the surgical approach. Patient age was 13 +/- 10 years, and accompanying diagnoses were: truncus arteriosus, subaortic stenosis, ventricular septal defect, and tetralogy of Fallot. Repeat transesophageal echocardiography was performed after each cardiopulmonary bypass run to determine residual AI and mechanism. Aortic valve leaflet number ranged from 2 to 4. AI was graded 2 to 4+, and postoperatively 0 to 2+. Primary mechanisms were: cusp prolapse (7), leaflet holes (4), restricted leaflet motion (2), and annular dilation (3). Patients required 1 to 3 cardiopulmonary bypass runs until primary AI mechanism was abolished. In all, 12 of 14 patients were free from death or repeated surgery at 2 years. Surgical repair of congenital AI may be aided by transesophageal echocardiographic guidance, with repeat short cardiopulmonary bypass as needed. Long-term studies are needed to determine durability of repair.

Nonsurgically-acquired complete atrioventricular block in endocardial cushion defect.

Complete atrioventricular heart block (CAVB) most commonly occurs as a complication of cardiac surgery. We report the development of CAVB in an 8-year-old girl with endocardial cushion defect (ECD) who had not undergone a cardiac operation. Although this is the first report of acquired non-surgical CAVB in a child with ECD, we believe that the development of CAVB in patients with unoperated ECD occurs more commonly than is usually realized. An increased awareness of this possibility will allow expedient diagnosis, which is essential to proper treatment. In most cases, the implantation of a permanent ventricular pacemaker will alleviate congestive heart failure or prevent sudden death.

SUCCESSFUL SURGICAL MANAGEMENT OF CONGENITAL ARTERIOVENOUS FISTULA OF THE SUBCLAVIAN ARTERY: CASE REPORT.

A 10-month-old infant was found to have an isolated congenital arteriovenous fistula between the right subclavian artery and vein. Ligation of the fistula resulted in complete anatomic correction and rapid resolution of cardiomegaly.