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.

Global left ventricular relaxation: A novel tissue Doppler index of acute rejection in pediatric heart transplantation.

BACKGROUND: Serial invasive cardiac catheterization with endomyocardial biopsies (EMBs) is the current standard of reference for evaluation after orthotopic heart transplant (OHTx). We developed a novel, non-invasive echocardiographic index of global left ventricular relaxation (LVRI) and assessed its sensitivity, specificity and predictive value for detecting rejection compared with EMB results in a prospective study conducted from September 2012 through May 2014. METHODS: LVRI was calculated as the sum of diastolic tissue Doppler imaging (TDI) velocities (E') of the left ventricular lateral, septal and posterior walls divided by the percentage of left ventricular posterior wall (LVPW) thinning by M-mode. LVRI was measured in 47 OHTx patients and 50 patients with normal hearts. Of the 33 patients who underwent clinically indicated EMB, 22 patients had Grade 0R EMB, 6 had Grade 1R and 5 had Grade 2R to 3R biopsy results. Sensitivity, specificity and predictive value of LVRI for discriminating Grade 1R to 3R EMB were calculated. The LVRI was compared before and after OHTx rejection treatment and during the early and late post-transplant period. To characterize LVRI, 1-way analysis of variance was used to compare all groups, including non-OHTx patients. RESULTS: LVRI was lower in patients with Grade 0R EMBs compared with non-OHTx patients. Patients with Grade 1R to 3R EMBs had lower LVRI than those with Grade 0R EMBs. LVRI recovered after treatment for rejection. LVRI appeared to normalize between 40 and 90 days post-transplantation. After 90 days, sensitivity was 100% and specificity was 90.9% for detecting patients with Grade 1R to 3R EMBs using an LVRI of 0.8. CONCLUSION: LVRI, a novel, non-invasive TDI index of global left ventricular diastolic dysfunction, appears to be useful for detecting rejection in children beyond 3 months post-OHTx.

Premature Atherosclerotic Cardiovascular Disease in Childhood Cancer Survivors.

Survival rates of childhood cancer have increased over the last 30 years, revealing a population with unique characteristics and risks. The effects of radiation and cardiotoxic chemotherapy predispose these children to both early and late cardiovascular disease. Cranial radiation also increases the likelihood of growth hormone deficiency, which leads to metabolic disturbances. Childhood cancer survivors are less likely to be active than their healthy siblings, and have a lower aptitude for physical activity. These issues are additive to the usual risks experienced by the general population, thereby significantly increasing the likelihood of premature cardiovascular disease. Early and regular screening and risk factor management in this population is recommended.

The Right Subclavian Artery Arising as the First Branch of a Left-Sided Aortic Arch.

We describe an unusual pattern of branching of a left-sided aortic arch in which the first branch is the right subclavian artery, followed by the common carotid arteries arising from a common trunk. The patient was born with transposition (concordant atrioventricular and discordant ventriculoarterial connections) and had 18q23 deletion. We discuss the implication of these anatomic findings in the light of inferences currently made by echocardiographers when the first branch of the aortic arch fails to bifurcate. We also relate the findings to the concepts of cardiac development and draw comparisons with previous descriptions, and interpretations of the morphogenesis, of the patterns of branching from the aortic arch.

Surgical Placement of Permanent Epicardial Pacing Systems in Very Low-Birth Weight Premature Neonates: A Review of Data From the Pediatric Cardiac Care Consortium (PCCC).

UNLABELLED: Few studies have characterized the surgical outcomes following epicardial pacemaker placement in very low-birth weight infants with congenital complete heart block. This study was undertaken to review the surgical experience with this patient population based on data from a large multi-institutional registry. METHODS: The Pediatric Cardiac Care Consortium (PCCC) multi-institutional database was retrospectively reviewed to identify premature, low-birth weight neonates that underwent surgical placement of an epicardial pacing system for heart block. We reviewed 179 patients with birth weights less than 1.5 kg that underwent a major operative procedure. Of these, 10 patients underwent surgical placement of an epicardial pacing system for heart block. Patients had heart block in otherwise structurally normal hearts (n = 6) or heart block associated with complex structural congenital cardiac anomalies (n = 4). RESULTS: There were no deaths directly related to the surgical placement of the epicardial pacing system. There were no immediate complications with either lead or generator placement. One generator pocket was revised three months following placement. Survival to discharge was 60%. The four deaths occurred at a mean of 11 days (range 1-45 days) following the procedure. CONCLUSIONS: Neonates born with prematurity and congenital heart block represent a challenging subset of patients with significant mortality. Generator pocket breakdown and infection have been considered barriers to optimal short- and long-term outcomes. Among cases in the PCCC, there were no deaths or major complications that could be attributed to permanent epicardial pacemaker placement. These data suggest that an aggressive surgical strategy may be justified.

Repair of major congenital cardiac defects in low-birth-weight infants: is delay warranted?

OBJECTIVES: Few studies have described the survival of low-birth-weight infants weighing less than 1.5 kg at operation for a cardiac malformation. Our goal was to determine if body weight at surgery affects survival. METHODS: This was a retrospective cohort study using outcome data from the Pediatric Cardiac Care Consortium between 1982 and 2006. RESULTS: We reviewed the outcomes of 450 consecutive infants with a cardiac anomaly and a birth weight of less than 1.5 kg, and weight of less than 2.5 kg at surgery. Of these, 179 patients had undergone surgery with a weight of less than 1.5 kg and 271 patients weighed 1.5 to 2.5 kg at surgery. The 30-day survival rate was 83% for cohort 1 and 86% for cohort 2. For patients not requiring cardiopulmonary bypass, the 30-day survival rate was 86% for cohort 1 and 92% for cohort 2. For patients requiring cardiopulmonary bypass, the 30-day survival rate was 69% for cohort 1 and 73% for cohort 2. No notable improvement in the outcomes occurred over time. CONCLUSIONS: For low-birth-weight infants (weight < 1.5 kg) undergoing a major cardiac procedure, the survival of infants weighing less than 1.5 kg at surgery is comparable to that of infants who weighed 1.5 to 2.5 kg. We conclude that, in our series, weight was not an independent risk factor for mortality, and, therefore, operative delay because of patient weight might be unwarranted.