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.

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.

ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography.

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and the Society of Pediatric Echocardiography to develop Appropriate Use Criteria (AUC) for multimodality imaging during the follow-up care of patients with congenital heart disease (CHD). This is the first AUC to address cardiac imaging in adult and pediatric patients with established CHD. A number of common patient scenarios (also termed "indications") and associated assumptions and definitions were developed using guidelines, clinical trial data, and expert opinion in the field of CHD.1 The indications relate primarily to evaluation before and after cardiac surgery or catheter-based intervention, and they address routine surveillance as well as evaluation of new-onset signs or symptoms. The writing group developed 324 clinical indications, which they separated into 19 tables according to the type of cardiac lesion. Noninvasive cardiac imaging modalities that could potentially be used for these indications were incorporated into the tables, resulting in a total of 1,035 unique scenarios. These scenarios were presented to a separate, independent panel for rating, with each being scored on a scale of 1 to 9, with 1 to 3 categorized as "Rarely Appropriate," 4 to 6 as "May Be Appropriate," and 7 to 9 as "Appropriate." Forty-four percent of the scenarios were rated as Appropriate, 39% as May Be Appropriate, and 17% as Rarely Appropriate. This AUC document will provide guidance to clinicians in the care of patients with established CHD by identifying the reasonable imaging modality options available for evaluation and surveillance of such patients. It will also serve as an educational and quality improvement tool to identify patterns of care and reduce the number of Rarely Appropriate tests in clinical practice.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome.

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy.

Utility of Multimodality Imaging in the Morphologic Characterization of Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Anomalous aortic origin of a coronary artery from the wrong Sinus of Valsalva (AAOCA) is a rare congenital anomaly and is associated with sudden cardiac death. Morphologic features considered to be "high risk" are significant luminal narrowing, acute coronary angulation at its origin, intramural course, and long interarterial course. A consistent approach for characterization of these features is lacking. METHODS: A retrospective single-center review of all patients diagnosed with AAOCA using echocardiogram and computed tomography (CT)/magnetic resonance imaging (MRI) studies was performed. Twenty-nine patients were identified (25 using CT and 4 using MRI) with subsequent three-dimensional data sets. The MRI data sets lacked adequate resolution and were excluded. Twenty-five patients (median age 15.1, range 10-39.5 years, 72% male) were further analyzed using echocardiogram and CT. Morphologic assessment focused on luminal stenosis, coronary angulation, and interarterial length. Additional morphologic features focusing on cross-sectional area and degree of ellipticity were also assessed. RESULTS: Echocardiography tended to yield smaller measurements compared to CT and had poor interobserver reproducibility for measurements pertaining to the narrowest proximal and distal coronary segments. Computed tomography showed good inter-/intraobserver reproducibility for the same. Agreement between both modalities for coronary angulation at its origin was excellent. There was good agreement for measurements of interarterial length between echocardiography and CT, but echocardiography had superior reproducibility. Assessment of luminal cross-sectional area and elliptical shape by CT had excellent inter-/intraobserver reproducibility. CONCLUSION: The combination of echocardiography and CT characterizes morphologic features of anomalous origin of the coronary artery more reliably than either modality alone.

Primary tetralogy of Fallot repair: predictors of intensive care unit morbidity.

BACKGROUND: Primary repair of tetralogy of Fallot has low surgical mortality, but some patients still experience significant postoperative morbidity. Our objectives were to review our institutional experience with primary tetralogy of Fallot repair, and identify predictors of intensive care unit morbidity. METHODS: We reviewed all patients with tetralogy of Fallot who underwent primary repair in infancy from 2001 to 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. Intensive care unit morbidity was defined as prolonged intensive care unit stay (≥ 7 days) and/or prolonged duration of mechanical ventilation (≥ 48 h). RESULTS: 97 patients who underwent primary surgical repair during the study period were included in the study. The median age was 4.9 months (range 1-9 months) and the median weight was 5.3 kg (range 3.1-9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median intensive care unit stay was 6 days (range 2-21 days) and the median duration of mechanical ventilation was 19 h (range 0-136 h). Age and weight were independent predictors of intensive care unit stay, while surgical era predicted the duration of mechanical ventilation. CONCLUSION: Primary tetralogy of Fallot repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery were significant predictors of morbidity.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Fetal diagnosis of left-ventricular noncompaction cardiomyopathy in identical twins with discordant congenital heart disease.

Left-ventricular noncompaction (LVNC) is characterized by prominent myocardial trabeculae with deep intratrabecular recesses. Associated with ventricular dysfunction, LVNC can present in isolation or in conjunction with structural heart defects (i.e., "nonisolated" LVNC). Although it was once considered to be difficult and unreliable, successful detection of this condition by way of fetal echocardiography has been well documented. We present what is to our knowledge the first prenatal diagnosis of LVNC in monochorionic-monozygotic twins. Nonisolated LVNC was identified in twins A and B at 23 weeks' gestation. A noncompaction-to-compaction ratio >2 was documented in both cases. Discordance for congenital heart disease was observed in the twins: Twin A presented with pulmonary atresia with an intact ventricular septum, critical tricuspid stenosis, and severe right-ventricular hypoplasia, whereas twin B presented with mild mitral valve dysplasia. Despite an initially complicated postnatal course for twin A, this case shows that it is feasible to achieve good outcomes with accurate prenatal detection and aggressive postnatal management.

Factors affecting the decision to defer endotracheal extubation after surgery for congenital heart disease: a prospective observational study.

BACKGROUND: Fast-tracking and early endotracheal extubation have been described in patients undergoing surgery for congenital heart disease (CHD); however, criteria for patient selection have not been validated in a prospective manner. Our goal in this study was to prospectively identify factors associated with the decision to defer endotracheal extubation in the operating room (OR). METHODS: We performed a prospective observational study of 275 patients (median age 18 months) at the Mount Sinai Medical Center (MSMC), New York, New York, and 49 patients (median age 25 months) at the University of Tokyo Hospital (UTH), Tokyo, Japan, undergoing surgery for CHD requiring cardiopulmonary bypass. These patients were all eligible for fast-tracking, including extubation in the OR immediately after surgery, according to the respective inclusion/exclusion criteria applied at the 2 sites. RESULTS: Eighty-nine percent of patients at the MSMC, and 65% of patients at the UTH were extubated in the OR. At the MSMC, all patients without aortic cross-clamp, and patients with simple procedures (Risk Adjustment for Congenital Heart Surgery [RACHS] score 1) were extubated in the OR. Among the remaining MSMC patients, regression analysis showed that procedure complexity was still an independent predictor for not proceeding with planned extubation in the OR. Extubation was more likely to be deferred in the RACHS score 3 surgical risk patients compared with the RACHS score 2 group (P = 0.005, odds ratio 3.8 [CI: 1.5, 9.7]). Additionally, trisomy 21 (P = 0.0003, odds ratio 9.9 [CI: 2.9, 34.5]) and age (P = 0.0015) were significant independent predictors for deferring OR extubation. We tested our findings on the patients from the UTH by developing risk categories from the MSMC data that ranked eligible patients according to the chance of OR extubation. The risk categories proved to predict endotracheal extubation in the 49 patients who had undergone surgery at the UTH relative to their overall extubation rate, despite differences in anesthetic regimen and inclusion/exclusion criteria. CONCLUSIONS: Preoperatively known factors alone can predict the relative chances of deferring extubation after surgery for CHD. The early extubation strategies applied in the 2 centers were successful in the majority of cases.

High prevalence of structural heart disease in children with cblC-type methylmalonic aciduria and homocystinuria.

OBJECTIVE: To characterize the frequency and nature of cardiovascular defects in patients with CblC-type methylmalonic aciduria and homocystinuria (cblC), an inborn error of cobalamin (vitamin B12) metabolism resulting in accumulation of methylmalonic acid and homocysteine. STUDY DESIGN: A retrospective observational study was conducted investigating 10 patients with cblC ranging in age from 2 weeks to 24 years (mean 4.4 years +/- 7.5 years, median 0.6 years). All patients underwent a complete 2-D echocardiogram including quantitative assessment of left ventricular systolic function. RESULTS: Structural heart defects were detected in 50% of patients with cblC. Heart defects included left ventricular (LV) non-compaction (3), secundum atrial septal defect (2), muscular ventricular septal defect (1), dysplastic pulmonary valve without pulmonary stenosis (1) and mitral valve prolapse with mild mitral regurgitation (1). One patient had resolved cor pulmonale and right heart failure secondary to pulmonary embolism. All patients had quantitatively normal LV systolic function. CONCLUSIONS: Diverse and clinically significant structural heart defects appear to be highly prevalent in cblC, perhaps due to abnormal DNA and histone methylation during embryogenesis. The specific cardiac defects detected in our cohort were variable, and studies with a larger number of patients are needed to establish which forms are most common. Routine and periodic cardiovascular evaluation may be indicated in patients with cblC.

Multisite near-infrared spectroscopy predicts elevated blood lactate level in children after cardiac surgery.

OBJECTIVES: To determine if a relationship exists between regional oxyhemoglobin saturation (rSO(2)) measured at various body locations by near-infrared spectroscopy (NIRS) and blood lactate level in children after cardiac surgery. DESIGN: A prospective, observational study. SETTING: A pediatric cardiac intensive care unit in a university hospital. PARTICIPANTS: Twenty-three children undergoing repair of congenital heart disease. Patients with single-ventricle physiology and/or residual intracardiac shunts were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cerebral, splanchnic, renal, and muscle rSO(2) values were recorded every 30 seconds via NIRS for 24 hours postoperatively. Blood lactate levels measured minimally at 0, 2, 4, 6 and 24 hours postoperatively were correlated with rSO(2) values derived by averaging all values recorded during the 60 minutes preceding the blood draw. Twenty-three patients were enrolled with 163 lactate measurements and more than 39,000 rSO(2) observations analyzed. Cerebral rSO(2) had the strongest inverse correlation with lactate level followed by splanchnic, renal, and muscle rSO(2) (r = -0.74, p < 0.0001, r = -0.61, p < 0.0001, r = -0.57, p < 0.0001, and r = -0.48, p < 0.0001, respectively). The correlation improved by averaging the cerebral and renal rSO(2) values (r = -0.82, p < 0.0001). Furthermore, an averaged cerebral and renal rSO(2) value or=3.0 mmol/L with a sensitivity of 95% and a specificity of 83% (p = 0.0001). CONCLUSIONS: Averaged cerebral and renal rSO(2) less than 65% as measured by NIRS predicts hyperlactatemia (>3 mmol/L) in acyanotic children after congenital heart surgery. Hence, this noninvasive, continuous monitoring tool may facilitate the identification of global hypoperfusion caused by low cardiac output syndrome in this population.

Extubation in the operating room after congenital heart surgery in children.

OBJECTIVE: Early extubation in the operating room after surgery for congenital heart disease has been described; however, postoperative mechanical ventilation in the intensive care unit remains common practice in many institutions. The goal of this study was to identify perioperative factors associated with not proceeding with planned operating room extubation. METHODS: We performed a retrospective chart review of 224 patients (aged 1 month to 18 years, median 20 months) undergoing surgery for congenital heart defects requiring cardiopulmonary bypass. Patients mechanically ventilated preoperatively were excluded. A stepwise logistic regression model was used to test for the independent influence of various perioperative factors on extubation in the operating room. RESULTS: Overall, 79% of patients were extubated in the operating room. Younger age and longer cardiopulmonary bypass time were the strongest predictors for not extubating. Each step down to a younger age group (<2, 2-4, 4-6, 6-12, >12 months) reduced the chance of extubation in the operating room by 56%. Cardiopulmonary bypass time for more than 150 minutes was associated with an 11.8-fold increased risk of not being extubated. Male gender and high inotrope requirement after cardiopulmonary bypass were also significantly associated with fewer children being extubated. CONCLUSION: Extubation in the operating room after surgery for congenital heart disease was successful in the majority of patients. The strongest independent risk factors for failure of this strategy included younger age and longer cardiopulmonary bypass time.

Echocardiographic diagnosis of clinically silent congenital coronary artery anomalies.

BACKGROUND: The incidence of congenital coronary anomalies is mainly derived from autopsy series and pre-participation exams in athletes. Limited data exist regarding the spectrum of coronary anomalies that can be detected in asymptomatic patients. We sought to describe echocardiographically detected congenital coronary artery anomalies in asymptomatic children after implementing a screening protocol mandating identification of coronary artery origin and proximal course in all initial studies. METHODS: Our database was searched from 1/1/1993 to 3/31/2006 and all echocardiograms coded for coronary anomalies were identified. Clinically "silent" congenital coronary anomalies were culled from that group. RESULTS: Of the 168 "silent" coronary anomalies detected, 111 were anomalies of aortic origin, including 59 patients with "high coronary takeoff" and 30 patients with "wrong sinus" origin of either the left or right coronary artery. Small coronary fistulas were seen in 57. Associated congenital heart defects were found in 53% of individuals with coronary anomalies. CONCLUSIONS: This study comprises the largest group of echocardiographically detected, "silent" but potentially clinically significant, congenital coronary anomalies in children. Prospective echocardiographic diagnosis of "high coronary takeoff", a risk factor for injury during cardio-pulmonary bypass, and asymptomatic intraseptal coronary stenosis are described for the first time.

Continuous positive airway pressure and lung separation during cardiopulmonary bypass to facilitate congenital heart surgery via the right thorax in children.

We describe a fast track anesthesia technique that facilitates congenital heart surgery via right axillary thoracotomy in children. Continuous positive airway pressure on the dependent lung, before and during cardiopulmonary bypass, approximates the heart towards the chest wall incision, and significantly improves the surgeon's access to the heart.

Description of a new technique for reimplanting the anomalous right pulmonary artery.

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital lesion with a high mortality and morbidity if early diagnosis is not made and correction is not undertaken. We describe the repair of such a lesion using a double-trapdoor technique of pulmonary artery reimplantation.