Jennifer C. Romano

Single Ventricle and Total Anomalous Pulmonary Venous Connection: Implications of Prenatal Diagnosis:

Background: Single ventricle (SV) patients with total anomalous pulmonary venous connection (TAPVC) are at high risk. Given the limited published data available, we examined outcomes and the implications of a prenatal diagnosis of SV/TAPVC. Methods: A single-center, retrospective review was performed in neonates with SV/TAPVC from 1998 to 2014, identified through institutional databases. Patient demographic, perioperative, and follow-up data were collected. Results: Thirty-four eligible infants with SV/TAPVC were identified (mean birth weight: 3.0 kg). The TAPVC types were supracardiac (59%), infracardiac (21%), mixed (12%), and cardiac (9%). Heterotaxy syndrome was present in 25 (74%) infants. A prenatal diagnosis of SV was made in 26 (76%) infants, with TAPVC identified in 12 (35%). Seventeen (50%) had obstructed TAPVC within the first 48 hours of life; 7 of these patients had obstructed TAPVC identified prenatally. There were two preoperative deaths. Overall survival for the cohort was 65% at 1 year and 50% at 3 years. Survival in the obstructed group was significantly worse compared to the unobstructed group (47% vs 81% at 1 year; 27% vs 73% at 3 years, P = .01). Obstructed TAPVC and a prenatal prediction of obstructed TAPVC were significantly associated with postoperative mortality (P = .01 and .03, respectively). Conclusions: Patients with SV/TAPVC remain a high-risk group, with obstructed TAPVC a significant risk factor for mortality. Prenatal diagnosis of TAPVC in SV patients is challenging, but given those with obstructed TAPVC are especially at high risk, improved prenatal diagnostic techniques in this group may enhance counseling/delivery planning.

Similar Interstage Outcomes for Single Ventricle Infants Palliated With an Aortopulmonary Shunt Compared to the Norwood Procedure

Background: Interstage outcomes for single ventricle infants following Norwood operation have been well studied, showing significant mortality. Other single ventricle infants require only an aortopulmonary shunt. The aim of the study was to describe the interstage outcomes of this group compared to Norwood patients and identify risk factors for mortality. Methods: A single-center retrospective cohort review was performed in patients who underwent a Norwood operation (Norwood) or aortopulmonary shunt (Shunt) during 2000 to 2011 and survived to discharge. Hybrid or pulmonary artery banding patients were excluded. Univariate comparison was made between Norwood and Shunt patients as well as a Shunt subgroup analysis. Results: A total of 486 patients (368 Norwood and 118 Shunt) were included. Norwood and Shunt patients were similar in terms of preterm birth, surgery weight, and stage 1 complications. Shunt patients were more likely to be female, have an extracardiac or genetic anomaly, and have a shorter hospital length of stay compared to the Norwood patients (all P < .0001). No significant difference in interstage mortality was seen between the Shunt and Norwood patients (6.8% vs 11.1%, respectively; P = .17). Stage 2 mortality was also similar (Shunt 4.6% vs Norwood 7.8%; P = .25). In the Shunt patients, those who died during interstage weighed less at surgery (2.7 [0.7] kg vs 3.3 [0.7] kg, P = .03) and were more likely to have arrhythmias (50% vs 12%, P = .01), compared to survivors. Conclusions: Shunt patients have an interstage mortality that is not significantly less than Norwood patients. Lower weight at surgery and arrhythmias are risk factors for interstage death in Shunt patients.

Development of a Congenital Heart Surgery Composite Quality Metric: Part 2 - Analytic Methods

BACKGROUND We describe the statistical methods and results related to development of the first congenital heart surgery composite quality measure. METHODS The composite measure was developed using The Society of Thoracic Surgeons Congenital Heart Surgery Database (2012 to 2015), Bayesian hierarchical modeling, and the current Society of Thoracic Surgeons risk model for case-mix adjustment. It consists of a mortality domain (operative mortality) and morbidity domain (major complications and postoperative length of stay). We evaluated several potential weighting schemes and properties of the final composite measure, including reliability (signal-to-noise ratio) and hospital classification in various performance categories. RESULTS Overall, 100 hospitals (78,425 operations) were included. Each adjusted metric included in the composite varied across hospitals: operative mortality (median, 3.1%; 10th to 90th percentile, 2.1% to 4.4%) major complications (median 11.7%, 10th to 90th percentile, 6.4% to 17.4%), and length of stay (median, 7.0 days; 10th to 90th percentile, 5.9 to 8.2 days). In the final composite weighting scheme selected, mortality had the greatest influence, followed by major complications and length of stay (correlation with overall composite score of 0.87, 0.69, and 0.47, respectively). Reliability of the composite measure was 0.73 compared with 0.59 for mortality alone. The distribution of hospitals across composite measure performance categories (defined by whether the 95% credible interval overlapped The Society of Thoracic Surgeons average) was 75% (same as expected), 9% (worse than expected), and 16% (better than expected). CONCLUSIONS This congenital heart surgery composite measure incorporates aspects of both morbidity and mortality, has clinical face validity, and greater ability to discriminate hospital performance compared with mortality alone. Ongoing efforts will support the use of the composite measure in benchmarking and quality improvement activities.

Development of a Congenital Heart Surgery Composite Quality Metric: Part 1 - Conceptual Framework

BACKGROUND Current pediatric and congenital heart surgery quality measures focus on operative mortality, and numerous stakeholders are interested in more comprehensive measures. This report describes the background, rationale, and conceptual framework related to the development of the first composite quality metric in the field. METHODS A multidisciplinary panel reviewed methodology and framework related to quality measurement and several composite quality measures across adult cardiac surgery and other fields. The panel subsequently developed methodology and selected measures for a congenital heart surgery composite measure and reviewed potential advantages and limitations. Individual measures considered for potential inclusion in the composite were reviewed within the context of Donabedians triad and the Institute of Medicine quality domains. Decisions were made through group consensus. RESULTS The final composite measure selected is comprised of two domains: (1) a mortality domain (operative mortality) and (2) a morbidity domain (the 6 major complications endorsed by The Society of Thoracic Surgeons and Congenital Heart Surgeons Society plus cardiac arrest, and postoperative length of stay). Potential advantages include the more comprehensive view of quality compared with mortality alone and improvements in discrimination of hospital performance through increasing the number of end points. Potential limitations include the lack of longer term outcomes and challenges related to case-mix adjustment. CONCLUSIONS We have applied and adapted conceptual framework and methodology related to composite quality measures across other fields to congenital heart surgery. The composite quality metric created is inclusive of both morbidity and mortality, and expands our view of quality in this patient population.

Keep it simple: Repair of atrioventricular valve regurgitation in patients with a single ventricle

From the Section of Congenital Heart Surgery, Departments of Cardiac Surgery and Pediatrics, Michigan Medicine, University of Michigan, Ann Arbor, Mich. Disclosures: Author has nothing to disclose with regard to commercial support. Received for publication Oct 17, 2017; accepted for publication Oct 27, 2017. Address for reprints: Jennifer C. Romano, MD, MS, Departments of Cardiac Surgery and Pediatrics, 11-733 C. S. Mott Children’s Hospital, SPC 4204, 1540 E Hospital Dr, Ann Arbor, MI 48109-4204 (E-mail: jhirsch@umich. edu). J Thorac Cardiovasc Surg 2017;-:1-2 0022-5223/

Historical Perspectives of The American Association for Thoracic Surgery: Aldo Castañeda

36.00 Copyright 2017 by The American Association for Thoracic Surgery https://doi.org/10.1016/j.jtcvs.2017.10.105

KOMMERELL DIVERTICULUM ACROSS THE AGES: DESTINED FOR DILATION?

From the Department of Cardiac Surgery, Section of Pediatric Cardiac Surgery, University of Michigan, Ann Arbor, Mich; and Children’s Hospital of Philadelphia, Philadelphia, Pa. Disclosures: Authors have nothing to disclose with regard to commercial support. Received for publication Oct 3, 2016; revisions received Oct 3, 2016; accepted for publication Oct 13, 2016. Address for reprints: Jennifer C. Romano, MD, MS, Department of Cardiac Surgery, Michigan Congenital Heart Center, 11-733 C.S. Mott Children’s Hospital, 1540 E Hospital Dr, SPC 4204, Ann Arbor, MI 48109-4204 (E-mail: jhirsch@umich.edu). J Thorac Cardiovasc Surg 2016;-:1-4 0022-5223/

Design and initial results of a programme for routine standardised longitudinal follow-up after congenital heart surgery

36.00 Copyright 2016 by The American Association for Thoracic Surgery http://dx.doi.org/10.1016/j.jtcvs.2016.10.012

Congenital Heart Surgery Case Mix Across North American Centers and Impact on Performance Assessment

Background: A Kommerell Diverticulum (KD) is the dilated proximal portion of an aberrant subclavian artery as a consequence of abnormal embryonic vascular development. Concern has arisen for aneurysmal KD dilation, dissection and rupture, with a trend toward invasive prophylactic intervention. This

Infant Motor Skills After a Cardiac Operation: The Need for Developmental Monitoring and Care

BACKGROUND With improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Childrens Hospital of Philadelphia (CHOP) and University of Michigan (UM). METHODS We included children undergoing benchmark operations of the Society of Thoracic Surgeons. Considerations regarding personnel, patient/parent engagement, funding, regulatory issues, and annual data collection are described, and initial follow-up rates are reported. RESULTS The present analysis included 1737 eligible patients undergoing surgery at CHOP from January 2007 to December 2014 and 887 UM patients from January 2010 to December 2014. Overall, follow-up data, of any type, were obtained from 90.8% of patients at CHOP (median follow-up 4.3 years, 92.2% survival) and 98.3% at UM (median follow-up 2.8 years, 92.7% survival), with similar rates across operations and institutions. Most patients lost to follow-up at CHOP had undergone surgery before 2010. Standardised questionnaires assessing burden of disease/quality of life were completed by 80.2% (CHOP) and 78.4% (UM) via phone follow-up. In subsequent pilot testing of an automated e-mail system, 53.4% of eligible patients completed the follow-up questionnaire through this system. CONCLUSIONS Standardised follow-up data can be obtained on the majority of children undergoing benchmark operations. Ongoing efforts to support automated electronic systems and integration with registry data may reduce resource needs, facilitate expansion across centres, and support multi-centre efforts to understand and improve long-term outcomes in this population.