Cognitive Biases in High-Stakes Decision-Making: Implications for Joint Pediatric Cardiology and Cardiothoracic Surgery Conference.

Extensive research has consistently demonstrated that humans frequently diverge from rational decision-making processes due to the pervasive influence of cognitive biases. This paper conducts an examination of the impact of cognitive biases on high-stakes decision-making within the context of the joint pediatric cardiology and cardiothoracic surgery conference, offering practical recommendations for mitigating their effects. Recognized biases such as confirmation bias, availability bias, outcome bias, overconfidence bias, sunk cost fallacy, loss aversion, planning fallacy, authority bias, and illusion of agreement are analyzed concerning their specific implications within this conference setting. To counteract these biases and enhance the quality of decision-making, practical strategies are proposed, including the implementation of a no-interruption policy until all data is reviewed, leaders refraining from immediate input, requiring participants to formulate independent judgments prior to sharing recommendations, explicit probability estimations grounded in base rates, seeking external opinions, and promoting an environment that encourages dissenting perspectives.

Left Ventricular Dysfunction Following Repair of Ventricular Septal Defects in Infants.

Left ventricular systolic dysfunction (LVSD) is frequently observed following repair of ventricular septal defects (VSD), although little is known about its incidence, time course, or risk factors. Among infants undergoing VSD repair, for postoperative LVSD, we sought to determine (1) incidence, (2) predictors, and (3) time to resolution. We queried our institution's surgical database for infants who underwent repair of isolated VSDs from November 2001 through January 2019. The primary outcome was postoperative LVSD, which was defined as a shortening fraction (SF) of <26% by M-mode. Postoperative echocardiograms were reviewed, and measurements were made using standard methods. Receiver operating characteristic analysis was generated to determine the preoperative left ventricular internal dimension (LVIDd) z-score most predictive of LVSD. Multivariable analysis was conducted to determine associations with LVSD; covariates in the model were weight percentile, genetic syndrome, preoperative diuretic, VSD type, and preoperative LVIDd z-score. Of the 164 patients who met inclusion criteria, 62 (38%) had postoperative LVSD. Fifty-eight (94%) of patients had resolution of LVSD within 9 months of surgery. Preoperative LVIDd z-score of >3.1 was associated with both an increased incidence of postoperative LVSD and prolonged time to resolution. Multivariable logistic regression analysis showed only preoperative LVIDd z-score was independently associated with postoperative LVSD. LVSD following VSD closure is common, but nearly all cases resolve by 9 months postoperatively. Elevated LVIDd prior to surgery is associated with postoperative LVSD. These data suggest VSD closure should be considered prior to the development of significant left ventricular dilation.

Mid-aortic syndrome diagnosed by transesophageal echocardiogram in a patient with dilated cardiomyopathy.

Mid-aortic syndrome is a rare condition characterised by segmental narrowing of the thoracoabdominal aorta. Here, we demonstrate a case of mid-aortic syndrome in a 30-month-old female who was diagnosed via transesophageal echocardiography after presenting with dilated cardiomyopathy and severe heart failure requiring placement of a left ventricular assist device.

Referring Provider Opinions of Pediatric Cardiology Evaluations Performed by Nurse Practitioners.

In the setting of physician shortages, nurse practitioner (NP) roles have evolved, with increasing independence across most healthcare settings. We sought to characterize referring clinician perceptions of NP-performed outpatient pediatric cardiology consultations. We electronically distributed to pediatric and family medicine physicians and NPs in Arkansas our 11-item survey assessing the acceptability of pediatric cardiology consultations being completed by an NP under varying circumstances. Circumstances included seven common referral indications, and the scale offered five answer choices ranging from "definitely unacceptable" to "definitely acceptable". A total of 292 of 1756 (16.6% response rate) referring clinicians responded to the survey. Overall, 57% of responses indicated that NP-completed pediatric cardiology evaluations were either definitely or probably unacceptable. Acceptability was varied by referral indication and referring clinician characteristics. Unacceptability of NP-completed pediatric cardiology evaluations was greatest among family medicine physicians (81%), pediatricians (66%), and clinicians working in solo or two-physician practices (77%) or community hospitals/clinics (71%). If NP evaluation of a murmur included required review with a cardiologist, the unacceptability rate dropped from 50 to 24% (p < 0.0001). Unacceptability was higher in physicians who do not work with NPs (69%) compared to those who do (60%) (pp < 0.0001). Many referring physicians were willing to send patients ≥ 100 miles to ensure evaluation by a pediatric cardiologist. Most referring physicians find pediatric cardiology evaluations performed by NPs to be unacceptable. Requisite review with a cardiologist improved acceptability of NP evaluations. Many referring physicians would send patients much farther to guarantee evaluation by a cardiologist.

The Sinotubular Junction-to-Aortic Annulus Ratio as a Determinant of Supravalvar Aortic Stenosis Severity.

Supravalvar aortic stenosis (SVAS) severity guides management, including decisions for surgery. Physiologic and technical factors limit the determination of SVAS severity by Doppler echocardiography and cardiac catheterization in Williams syndrome (WS). We hypothesized SVAS severity could be determined by the sinotubular junction-to-aortic annulus ratio (STJ:An). We reviewed all preintervention echocardiograms in patients with WS with SVAS cared for at our center. We measured STJ, An, peak and mean Doppler gradients, and calculated STJ:An. We created 2 mean gradient prediction models. Model 1 used the simplified Bernoulli's equation, and model 2 used computational fluid dynamics (CFD). We compared STJ:An to Doppler-derived and CFD gradients. We reviewed catheterization gradients and the waveforms and analyzed gradient variability. We analyzed 168 echocardiograms in 54 children (58% male, median age at scan 1.2 years, interquartile range [IQR] 0.5 to 3.6, median echocardiograms 2, IQR 1 to 4). Median SVAS peak Doppler gradient was 24 mm Hg (IQR 14 to 46.5). Median SVAS mean Doppler gradient was 11 mm Hg (IQR 6 to 21). Median STJ:An was 0.76 (IQR 0.63 to 0.84). Model 1 underpredicted clinical gradients. Model 2 correlated well with STJ:An through all severity ranges and demonstrated increased pressure recovery distance with decreased STJ:An. The median potential variability in catheterization-derived gradients in a given patient was 14.5 mm Hg (IQR 7.5 to 19.3). SVAS severity in WS can be accurately assessed using STJ:An. CFD predicts clinical data well through all SVAS severity levels. STJ:An is independent of physiologic state and has fewer technical limitations than Doppler echocardiography and catheterization. STJ:An could augment traditional methods in guiding surgical management decisions.

Cutting balloon angioplasty on branch pulmonary artery stenosis in pediatric patients.

OBJECTIVES: To identify medium-term results following cutting balloon angioplasty (CBA) for branch pulmonary artery stenosis (PAS) and predictors of successful intervention. BACKGROUND: CBA has emerged as a successful alternative therapy for PAS resistant to conventional balloon angioplasty techniques but there is little information on medium and long-term outcomes. METHODS: This is a descriptive, single center, retrospective chart review of pediatric patients who underwent CBA for PAS at Arkansas Children's Hospital between May 2005 and December 2020. We reviewed demographics, procedural specifics, and 30-day complications. RESULTS: Forty-four patients underwent pulmonary artery CBA on 114 pulmonary artery segments through 126 catheterization cases, totaling 148 CBA events. Thirty-three individual pulmonary arteries underwent repeat intervention. Average minimal luminal diameter increase from pre-CBA to end of follow-up was 57% (CI, 38%-75%). Absence of Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) and the absence of Alagille Syndrome, Williams Syndrome, or Arterial Tortuosity Syndrome (ATS) were associated with increased odds of sustained success by 70% (CI, 0.11-0.79) and 91% (CI, 0.02-0.56), respectively. Increasing the cutting balloon diameter-to-minimal luminal diameter ratio by 0.5 increased odds of successful intervention by 2.37-fold (CI, 1.7-3.3). Seven patients had 30-day complications including one death. CONCLUSIONS: In the longest follow-up to date of children and adolescents who underwent CBA for branch PAS, we found that there was moderate medium-term success. Additionally, absence of TOF/PA/MAPCAs, absence of Alagille Syndrome, Williams Syndrome, or ATS, and high cutting balloon diameter-to-minimal luminal diameter ratio are predictors of sustained results.

Making the Match: Three Program Directors' Thoughts on Successfully Matching into Pediatric Cardiology Fellowship.

Successfully matching into a pediatric cardiology fellowship program is difficult, and there is currently a paucity of helpful material for prospective applicants. To address this gap and to guide pediatric cardiology fellowship applicants in navigating this process, three fellowship program directors have compiled a list of recommendations on how residents can improve their chances for a successful match. Detailed descriptions of the following recommendations are provided: become a great pediatrician, show consistent interest in pediatric cardiology, complete a research project, ensure great letters of recommendation, don't botch the personal statement, and apply wisely and interview effectively.

Circumaortic double left innominate vein: a rare echocardiographic diagnosis confirmed during congenital heart surgery.

We report a case of a newborn infant with coarctation of the aorta and hypoplastic transverse aortic arch who was found to have a circumaortic double left innominate vein on echocardiography. This exceedingly rare finding was important for surgical planning and was confirmed during congenital heart surgery.

Aortic Geometry in Patients with Duplication 7q11.23 Compared to Healthy Controls.

The aim of this study was to compare the size and geometry of the aorta in patients with 7q11.23 duplication (Dup7) to healthy controls. We retrospectively reviewed all echocardiograms in all patients with Dup7 evaluated at our institutions from June 2017 through September 2019. All standard aortic diameter measurements were made and recorded. Z-scores for the measurements were calculated. For comparison, a set of control echocardiograms was developed by randomly selecting 24 normal echocardiograms in age-matched patients who had undergone echocardiograms for an indication of either chest pain or syncope. In 58 echocardiograms from 21 Dup7 patients, all aortic measurements were increased compared to controls (p < 0.0001). Effacement of the sinotubular junction (STJ) of the aorta was present in all Dup7 patients. Our novel STJ-to-aortic annulus ratio of ≥ 1.15 had a 98.28% sensitivity (95% CI 90.76-99.96) and 100% specificity (95% CI 85.75-100) for distinguishing Dup7 from controls with a positive predictive value of 100% and a negative predictive value of 96.00% (95% CI 77.47-99.41). All patients in our study with Dup7 had echocardiographic evidence of aortopathy. Effacement of the STJ was present in all Dup7 patients. The STJ-to-annulus ratio is a better indicator of aortopathy in Dup7 than the aortic Z-score.

Real-Time Transthoracic Vector Flow Imaging of the Heart in Pediatric Patients.

In children with congenital heart defects, Doppler ultrasound is the standard, bedside imaging modality. However, precise characterization of blood flow is challenging due to angle-dependent and one-dimensional velocity estimation. Contrast agent free Vector Flow Imaging is a new ultrasound technology that enables angle-independent visualization of the detailed flow field. Two piglets, one with normal cardiac anatomy and one with congenital heart disease comprised of valvular pulmonary stenosis, a dilated main pulmonary artery, and an incomplete atrioventricular canal defect, were imaged transthoracically and epicardially using a BK Ultrasound bk5000 with built-in vector flow imaging and a 5MHz linear probe. Subsequently, two children, one with normal cardiac anatomy and one with congenital heart disease comprised of aortic valve stenosis and coarctation of the aorta were imaged transthoracically. Transthoracic two-dimensional echocardiography and vector flow imaging were readily performed in both animals and were limited only by the geometry of the porcine thorax. In addition, transthoracic vector flow imaging was successfully performed in both children, and abnormal flow secondary to cardiac anomalies was visible. Adequate penetration was obtained to a depth of 6.5 cm. Our group has previously demonstrated for the first time that transthoracic vector flow imaging echocardiography is feasible and practicable in pediatric-sized patients, and this paper describes examples of these concepts and in-depth comparisons with traditional imaging modalities. This paper demonstrates that commercially available vector flow imaging technology can be utilized in pediatric cardiac applications as a bedside transthoracic imaging modality, providing advanced detail of blood flow patterns within the cardiac chambers, across valves, and in the great arteries.

The Limited Benefit of Follow-Up Echocardiograms After Repair of Tetralogy of Fallot.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, making up 10% of all congenital heart defects. Annual follow-up echocardiograms are recommended in patients with repaired ToF, but evidence-based guidelines do not exist. We hypothesized that most echocardiograms performed in asymptomatic patients with repaired ToF and no physical exam change do not result in an actionable change (AC) in management. We retrospectively reviewed records of all patients with ToF and prior complete repair at our institution between January 2000 and September 2015. Changes in echocardiograms resulting in hospital admission, medication addition/change, cardiac catheterization, or surgical procedure were identified via chart review. These changes were referred to as an AC. A total of 1135 echocardiograms were reviewed from 233 patients (160 with initial complete repair, 70 with prior shunt, and 3 with other initial surgery). The median number of echocardiograms per patient was 5. Of the 1135 echocardiograms, 15 (1.3%) were associated with AC. Of the 15 patients with AC echocardiograms, 9 underwent a shunt prior to complete repair (9/70, 12.9%) and 6 had undergone an initial complete repair (6/160, 3.8%). The median age at AC was 6.3 years (IQR 4.4, 6.8) in the shunt group and 0.90 years (IQR 0.87, 1.1) in the initial complete repair group. In asymptomatic patients with repaired ToF and no physical exam change, echocardiograms rarely lead to a change in clinical management. In conclusion, the likelihood and timing of AC echocardiograms and reinterventions vary based on the type of initial surgery.

Learning strategies among adult CHD fellows.

OBJECTIVE: Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows. METHODS: This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible. RESULTS: A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as "very helpful", while topic-based teaching was considered "helpful" (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work. CONCLUSIONS: Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources.

Magnetocardiographic identification of prolonged fetal corrected QT interval in women receiving treatment for opioid use disorder.

AIM: Pregnant women undergoing treatment for opioid use disorder (OUD) may be exposed to multiple QT prolonging agents. We used magnetocardiography to measure fetal QT intervals in mothers with OUD on buprenorphine therapy. METHODS: Fetal and maternal magnetocardiography was performed in pregnant women receiving buprenorphine-assisted treatment (Disorder group); these were matched by gestational age to pregnant women who were opiate naïve (Reference group). Corrected QT intervals were determined using Bazett's formula and compared between groups. RESULTS: A total of eight women in the Disorder group matched to eight in the Reference group. Seven of the mothers (88%) in the Disorder group were smokers; there were no smokers in the Reference group. The average fetal corrected QT was significantly longer (P = 0.022) in the Disorder group than that in the Reference group (505 milliseconds [ms] ± 68.6 [standard deviation] vs 383 ms ± 70.3 [standard deviation]). CONCLUSION: Novel data from this small sample demonstrate prolongation of fetal corrected QT in women with OUD participating in buprenorphine assisted therapy. Additional investigation from a larger sample is needed to clarify if fetal buprenorphine and/or tobacco exposure is associated with changes in fetal QT which would warrant further prenatal and postnatal testing.

Comparison of inhospital outcomes of pediatric heart transplantation between single ventricle congenital heart disease and cardiomyopathy.

Data investigating the impact of household income and other factors on SV patient status-post-Fontan palliation after heart transplantation are lacking. We aim to evaluate factors affecting outcomes after OHT in this population. The PHIS database was interrogated for either SV or myocarditis/primary CM who were 4 years or older who underwent a single OHT. There were 1599 patients with a median age of 13.2 years (IQR: 9.3-16.1). Total hospital costs were significantly higher in the SV group ($408 000 vs $294 000, P < 0.0001), but as median household income increased, the risk of inhospital mortality, post-transplant LOS, and LOS-adjusted total hospital costs all decreased. The risk of inhospital mortality increased 6.5% per 1 year of age increase at the time of transplant. Patients in the SV group had significantly more diagnoses than those in the CM group (21 vs 15, P < 0.0001) and had longer total hospital LOSs as a result of longer post-transplant courses (25 days vs 15, P < 0.0001). Increased median household income and younger age are associated with decreased resource utilization and improved inhospital mortality in SV CHD patients who undergo OHT. In conclusion, earlier consideration of OHT in this population, coupled with improved selection criteria, may increase survival in this population.