Lindsay S. Rogers

18 years of the Fontan operation at a single institution: Results from 771 consecutive patients

OBJECTIVES The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients. BACKGROUND Since the initial description by Fontan, mortality associated with the Fontan operation has declined substantially. However, postoperative effusions remain a significant challenge. Effusions are a key determinant of postoperative length-of-stay and have been shown to be associated with the development of protein-losing enteropathy and with decreased survival. METHODS This study was a single-center, retrospective review of 771 patients who underwent Fontan palliation from 1992 to 2009. RESULTS Patients were divided into 3 eras dictated by shift in clinical practice. Overall mortality was 3.5%, 1% since 1996. Importantly, age at Stage II palliation decreased from Era 1 to Era 3 (7.1 vs. 5.9 months; p = 0.0001), whereas age at Fontan increased (1.7 vs. 2.8 years; p = 0.0001). The proportion of patients with prolonged hospital stay (46.7% vs. 8.2% vs. 19.5%, p < 0.001) decreased substantially after Era 1. A diagnosis of hypoplastic left heart syndrome and longer operative support times were associated with prolonged pleural drainage (odds ratio [OR]: 2.17, p < 0.001; OR: 1.2, p = 0.001) and hospital stay (OR: 1.48, p = 0.05; OR: 1.34, p < 0.001). In patients who underwent invasive assessment, higher pulmonary artery pressure was associated with death (OR: 1.37, p < 0.001) and prolonged hospital stay (OR: 1.09, p = 0.019). Pulmonary arterial pressure ≥15 mm Hg was 90% specific for discriminating unfavorable outcomes. CONCLUSIONS Mortality in the modern era is rare, whereas postoperative pleural drainage remains the dominant morbidity. Elevated pulmonary artery pressure seems to be a marker of unfavorable outcome. Continued investigation is warranted to determine whether medical interventions or alterations to operative strategy can alter peri-operative results and improve long-term outcomes.

Long-term survival after the Fontan operation: Twenty years of experience at a single center

Objective: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow‐up. This study aimed to describe long‐term post‐Fontan survival in a modern patient cohort. Methods: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. Results: Follow‐up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%‐95%), 90% at 10 years (95% CI, 88%‐92%), 85% at 15 years (95% CI, 82%‐88%), and 74% at 20 years (95% CI, 67%‐80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post‐Fontan ICU stay >1 week (HR, 2.4; P < .001). Conclusions: Long‐term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long‐term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.

Management of early Fontan failure: a single-institution experience

OBJECTIVE To analyse the incidence and outcomes of early Fontan failure (EFF) in a large contemporary cohort of palliated patients. METHODS A retrospective, single-centre study of all patients undergoing primary Fontan from 1 July 1995 to 31 December 2009 was performed. EFF was defined as death, need for extracorporeal membrane oxygenation (ECMO), Fontan takedown to superior cavopulmonary connection (SCPC) or transplantation within 30 days of the Fontan procedure. The incidence and outcomes were summarized with descriptive statistics, and risk factors for EFF were identified. RESULTS A total of 592 patients underwent primary Fontan procedure during the study period; 67% had a dominant right ventricle. An extracardiac conduit (ECC) was used for Fontan completion in 60.5%, with the remainder having a lateral tunnel. EFF occurred in 11 patients (1.9%), all of whom had ECC. ECMO was used in 5 patients, 5 had Fontan takedown and 2 had heart transplantation. Five of eleven, or 46%, study subjects died as opposed to an overall mortality for primary Fontan of 0.8%. Among patients who had Fontan takedown to SCPC, long-term survival was 80%. By univariate analysis, elevated ventricular end-diastolic pressure (9.5 ± 3.3 vs 7.4 ± 2.7 mmHg, P = 0.019) and total circulatory support time (99 ± 33 vs 71 ± 23 min, P = 0.001) were risk factors for EFF. The mean follow-up for the 6 hospital survivors was 5.9 years. There was one late transplant-related death. Of the 4 surviving patients who had Fontan takedown to a SCPC, 3 underwent subsequent Fontan completion and 1 underwent biventricular repair. CONCLUSIONS EFF is rare in the current era, but is associated with significant mortality. High filling pressures and a prolonged intraoperative course are risk factors for EFF. Of the management strategies available, Fontan takedown to an intermediate pathway appears to be associated with the best outcomes.

Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative

Background— Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies. Methods and Results— Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th–75th, 2–5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1–10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28–091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89–0.99; P=0.027), and ⩽mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04–6.30; P<0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15–0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00–1.15; P=0.057) and ⩽mild TR (HR, 3.50; 95% CI, 1.75–7.0; P<0.001). Number of reinterventions was associated with ⩽mild TR (rate ratio, 1.87; 95% CI, 1.23–2.87; P=0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ⩽mild TR (odds ratio, 18.6; 95% CI, 5.3–65.2; P<0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72–0.91; P<0.001). Conclusions— Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ⩽mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.

Assessment of the need for a cardiac morphology curriculum for paediatric cardiology fellows

BACKGROUND Expert knowledge of cardiac malformations is essential for paediatric cardiologists. Current cardiac morphology fellowship teaching format, content, and nomenclature are left up to the discretion of the individual fellowship programmes. We aimed to assess practices and barriers in morphology education, perceived effectiveness of current curricula, and preferences for a standardised fellow morphology curriculum. METHODS A web-based survey was developed de novo and administered anonymously via e-mail to all paediatric cardiology fellowship programme directors and associate directors in the United States of America; leaders were asked to forward the survey to fellows. RESULTS A total of 35 directors from 32 programmes (51%) and 66 fellows responded. Curriculum formats varied: 28 (88%) programmes utilised pathological specimens, 25 (78%) invited outside faculty, and 16 (50%) utilised external conferences. Director nomenclature preferences were split - 6 (19%) Andersonian, 8 (25%) Van Praaghian, and 18 (56%) mixed. Barriers to morphology education included time and inconsistent nomenclature. One-third of directors reported that <90% of recent fellow graduates had adequate abilities to apply segmental anatomy, identify associated cardiac lesions, or communicate complex CHD. More structured teaching, protected time, and specimens were suggestions to improve curricula. Almost 75% would likely adopt/utilise an online morphology curriculum. CONCLUSIONS Cardiac morphology training varies in content and format among fellowships. Inconsistent nomenclature exists, and inadequate morphology knowledge is perceived to contribute to communication failures, both have potential patient safety implications. There is an educational need for a common, online cardiac morphology curriculum that could allow for fellow assessment of competency and contribute to more standardised communication in the field of paediatric cardiology.

Response to Commentary: “How badly do we need instruction in paediatric cardiac anatomy?”

WE THANK DR ANDERSON FOR HIS INTEREST and thoughtful commentary on our article entitled “Assessment of the need for a cardiac morphology curriculum for paediatric cardiology fellows”. We appreciate his insights into the execution of the curriculum on which we are embarking on the basis of this published needs assessment. We also hope that this endeavour will have implications beyond the United States’ training programmes, although for simplicity’s sake our original needs assessment focussed on fellowship education within the United States training programmes. Overall, we agree that this effort will require collaboration and assistance from those with interest and expertise in education and cardiac morphology, and we welcome anyone interested to get in touch with the authors directly. We specifically address some points brought forth by Dr Anderson in his commentary. As our study demonstrates, the need for a common language is imperative to communication between providers. The work by the International Nomenclature Committee for Pediatric and Congenital Heart Disease serves as a basis for a common language and will certainly provide a scaffold for our curriculum. Dr Anderson goes on to describe that there are more similarities than previously understood between the two commonly used nomenclature systems – “Andersonian” and “Van Praaghian”. These similarities and differences are brought together nicely by Dr Ezon et al in their recent atlas.We agree that the last decade has brought some consensus between the two systems. Important differences, however, continue to exist – for example, the simple classification of double-outlet right ventricle is not consistent between the two systems. Some hearts classified as Tetralogy of Fallot, secondary to mitral-to-aortic valve fibrous continuity, with the aorta assigned to the left ventricle under the Van Praagh system are called double-outlet right ventricle by the Anderson classification system secondary to the degree of aortic override (>50% over the right ventricle, despite the aortic/mitral relationship). Therefore, understanding the major features and classification schemes that govern both systems is imperative to understanding the meaning of their language. Given the widespread use of both systems, especially within the United States of America, the only way to fully prepare fellows to be successful communicators within the current paediatric cardiology community is to familiarise them with both major classification systems – in essence, making them bilingual. Although it is our hope that the International Classification of Disease (ICD-11) classifications will serve as a common language moving forward, this change will take time and considerable effort in spreading this language, the details of which are still being debated within the nomenclature committee. Therefore, although ideally our proposed curriculum would present one unified way of speaking, we think the reality of current practice necessitates our learners understanding both major systems, using the ICD-11 terminology as a guide. Consistent with the principles of adult learning theory and appealing to the learning styles of our Correspondence to: L. S. Rogers, Division of Pediatric Cardiology, Children’s Memorial Herman Hospital, UT Health, 6410 Fannin Street, Suite 425, Houston, TX 77030, United States of America. Tel: +1 713 500 5737; Fax: +1 713 500 5751; E-mail: lindsay.s.rogers@uth.tmc.edu Cardiology in the Young 2017; Page 1 of 2

BEYOND THE FONTAN: SURGICAL AND CATHETER BASED REINTERVENTIONS ARE COMMON IN LONG-TERM SINGLE VENTRICLE SURVIVORS

The Fontan operation is the final planned stage of surgical palliation for children with single ventricle physiology. The burden of subsequent surgical and catheter-based reintervention is not well described in the modern Fontan population. Available medical records were reviewed for all patients

P08.13: Aortic outflow hypoplasia with dilated left ventricle and severe mitral insufficiency: the mitral valve dysplasia syndrome

in imaging protocols. The detection rates in in-born babies was considerably higher than in out-born babies. There was close correlation between antenatal and post-natal findings. Conclusions: We have demonstrated an improvement in overall detection rates over a 10 year period, however rates are still not close to 100%. The next step is to implement an educational plan for our rural and regional hospitals and hope to then demonstrate a further improvement in detection levels, in order to reduce the incidence or out-born babies.