Imaging of Double Inlet Left Ventricle.

Double inlet left ventricle (DILV) is a form of single ventricle heart disease where both atrioventricular valves enter a single left ventricle. Surgical intervention may be needed in the neonatal period secondary to systemic outflow tract obstruction or less commonly pulmonary obstruction. Two-dimensional echocardiography can adequately assess newborn anatomy and define the need for surgery. Beyond the newborn period, there is a renewed interest in septation of DILV using intracardiac baffles in a staged approach. Cross sectional imaging can aid in surgical planning. This article will review common anatomic features of DILV and imaging considerations for both single ventricle palliation and DILV septation.

Double outlet right ventricle - the 50% rule has always been about the conus.

PURPOSE OF REVIEW: There has been much variability in the definition of double outlet right ventricle (DORV) spanning the last century. Historically, emphasis has been placed on the assignment of the great arteries to the right ventricle as a definition of DORV. In this review, we aim to underscore the importance of conal muscle, rather than rules surrounding assignment of great arteries to ventricles. We will be outlining the variability in patient anatomy that results from variations in conal muscle development in DORV, which may not fit perfectly into predefined constructs. This anatomic variability directly determines physiology and surgical repair options. RECENT FINDINGS: There is a growing appreciation of the utility of cross-sectional imaging in complex DORV, and the generation of patient-specific 3D models with virtual reality simulations for surgical planning. These models improve the prediction of candidacy for biventricular repair and allow the mapping of complex baffle pathways preoperatively. SUMMARY: DORV is not a disease entity in itself, but rather a vast spectrum of disorders associated with maldevelopment of conal muscle and often abnormal expansion of one the great vessels. Patient-specific 3D models will be crucial for improved surgical planning and patient outcomes.

Hereditary haemorrhagic telangiectasia and SMAD4 mutation in a patient with complex single ventricle heart disease.

We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient's staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.

Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects.

BACKGROUND: To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications. METHODS: Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used. RESULTS: Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model. CONCLUSIONS: Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.

Sustained improvement in fellows' echocardiographic completeness through the coronavirus pandemic with a standardised imaging protocol.

First-year cardiology fellows must quickly learn basic competency in echocardiography during fellowship orientation. This educational process was disrupted in 2020 due to the coronavirus pandemic, as our hands-on echocardiography teaching transitioned from practice on paediatric volunteers to simulation-based training. We previously described an improvement in echocardiographic completeness after implementation of a standardised imaging protocol for the performance of acute assessments of ventricular function. Herein, we assessed whether this improvement could be sustained over the two subsequent years, including the fellowship year affected by the pandemic. Echocardiograms performed by first-year paediatric cardiology fellows to assess ventricular function were reviewed for completeness. The frequency with which each requested component was included was measured. A total demographic score (out of 7) and total imaging score (out of 23) were calculated. The pre-protocol years (2015-2017) were compared to the post-protocol years (2018-2020), and the pre-COVID years (2018-2019) were compared to the year affected by COVID (2020). There was a sustained improvement in completeness after protocol implementation with improvement in the demographic score (median increasing from 6 to 7, p < 0.001) and imaging score (median increasing from 13 to 16, p < 0.001). More individual components showed a statistically significant increase in frequency compared to our prior publication. The COVID pandemic resulted in very few differences in completeness. Demographic reporting improved modestly (p = 0.04); the imaging score was unchanged (p = 0.59). The only view obtained less frequently was the apical two-chamber view. A standardised imaging protocol allowed sustained improvements in echocardiographic completeness despite the disruption of fellowship orientation by COVID-19.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Medical education in pediatric and congenital heart disease: A focus on generational learning and technology in education.

Medical education is a complex interplay between teacher and trainee with the ultimate goal of producing competent physicians who provide excellent patient care. Physician education has evolved over centuries, from the apprenticeship of barber-surgeon through generations of bedside teachers and now evolving use of technology based instruction. All of these educational practices are based on expert assessment of effective techniques for imparting experience and knowledge to a new group of learners, the young doctor. In the past several decades, exponential growth in both medical innovation and technology development has occurred, leaving the current landscape of medical education with a substantial amount of medical data as well as innovative platforms for information access and distribution. These rapid changes have led to stark differences between medical educators and learners in their world views and preferences relating to teaching and learning. Therefore, understanding how the current generation of medical trainees perceives the world, accesses and retains information is imperative to effective education. The concept of generational learning can be used as a framework to identify teaching and learning preferences and help build relevant and effective educational content. This review article aims to outline our current understanding of generational characteristics, learning styles, and preferences. Using this framework, we will explore innovative educational content relevant to pediatric cardiology. Finally, we propose that a methodical approach to curriculum development will forge this generational gap and lead to even more effective and sharable educational content within our field.

Recent advances in our understanding of neurodevelopmental outcomes in congenital heart disease.

PURPOSE OF REVIEW: Patients with congenital heart disease (CHD) suffer from a pattern of neurodevelopmental abnormalities including deficits in language and executive function. In this review, we summarize recent studies that examine these outcomes, their risk factors, possible biomarkers, and attempts to develop therapeutic interventions. RECENT FINDINGS: The latest literature has highlighted the role of genetics in determining neurologic prognosis, as we have increased our understanding of potentially modifiable perioperative risk factors. The role of potentially neurotoxic medical therapies has become more salient. One recent focus has been how neurodevelopment affects quality of life and leads to a high prevalence of mental illness. Neuroimaging advances have provided new insights into the pathogenesis of deficits. SUMMARY: Although many risk factors in CHD are not modifiable, there is promise for interventions to improve neurodevelopmental outcomes in patients with CHD. Biomarkers are needed to better understand the timing and prognosis of injury and to direct therapy. Research into psychosocial interventions is urgently needed to benefit the many survivors with CHD.

A standardized imaging protocol improves quality and reduces practice variability in echocardiographic assessment of ventricular function by first-year pediatric cardiology fellows.

BACKGROUND: Echocardiography education for pediatric cardiology fellows has been a recent focus leading to the implementation of "boot camps." Less is described about continuing education through fellowship and improving image quality. We noticed practice variation in echocardiograms assessing ventricular function performed on nights and weekends. Thus, we implemented a standardized protocol and assessed its impact on imaging and reporting completeness. METHODS: We created an imaging protocol for the assessment of ventricular function in the acute setting. The protocol included demographic information, a list of images to be obtained, and the methods to quantify ventricular function. The protocol was explained to first-year fellows and distributed on an electronic quick reference card. Echocardiograms independently performed by first-year fellows during their first 4 months of on-call time were assessed pre- and postintervention using a standard rubric. RESULTS: Compliance with demographic reporting was high pre- and postintervention, but significantly improved after the standardized protocol (P < 0.001). Use of the protocol increased the median number of unique images obtained per echocardiogram from 13 to 17 (out of 23 required views, P < 0.001). Particularly improved was the performance of quantitative evaluations of function, including Simpson's method for left ventricular ejection fraction (four chamber: 40% vs 67%, P < 0.001; two chamber: 33% vs 67%, P < 0.001) and tricuspid annular plane systolic excursion (45% vs 80%, P < 0.001). CONCLUSIONS: The introduction of a standardized imaging protocol and its distribution to first-year fellows resulted in improvements in echocardiographic reporting completeness and increased the quality of information obtained by providing more quantitative assessments of ventricular function.

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study.

BACKGROUND: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. METHODS: We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1-year or prior to Glenn circulation). RESULTS: Eighty-one patients were included. At last follow-up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left-to-right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z-score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm2 /m2 had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm2 /m2 at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm2 /m2 included larger TV z-score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002). CONCLUSIONS: Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume-loading conditions signal increased capacity for growth sufficient for 2V circulation.

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P<0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01). CONCLUSIONS: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.

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.

Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (interquartile range 1.4 to 11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p = 0.001), pleural drainage >14 days (HR 1.5, 95% CI 1.01 to 2.2, p = 0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06 to 1.3, p = 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p <0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.

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.

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.