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Coarctation of the aorta, a stenotic disease of the juxtaductal aorta, causes upper extremity hypertension, lower extremity hypoperfusion, and various degrees of left ventricular hypertrophy or left ventricular failure depending on the tightness of the coarcted segment, the age at presentation, and associated cardiac lesions. Patients with coarctation of the aorta can be categorized into three major groups: (1) Patients with isolated coarctation (the greater majority), which can be associated with a bicuspid aortic valve; (2) Patients with coarctation and ventricular septal defect (VSD); (3) Patients who have associated complex heart disease, including truncus arteriosus, transposition of the great arteries, double outlet ventricles, or heterotaxy syndrome.

The Editorial Board of Cardiology in the Young has recently discussed the need for a Bioethics Forum and has given authorisation to proceed with its creation. Herein, we provide the organisational structure and launch process to introduce properly this interesting and timely endeavour. By this communication, we are establishing this Bioethics Forum of Cardiology in the Young. We hope to attract manuscripts concerning timely bioethical subjects and to offer the readership the opportunity to respond to these topics with supporting or opposing views as appropriate. New articles regarding timely topics will be written by the readership, as well as by invited authors, and these articles will be published. We hope to stimulate interactive discussion concerning the published manuscripts, and these manuscripts and the associated discussions will be open to all interested parties.

The Editorial Board of

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.

This article reviews the collaboration between clinician and illustrator throughout the ages while highlighting the era of cardiac surgery. Historical notes are based on Professor Sanjib Kumar Ghosh’s extensive review, literature searches, and the archives of the Johns Hopkins University Department of Art as related to Medicine in Baltimore. Personal communications were explored with medical illustrators and medical practitioners, many of whom are colleagues and trainees, to further chronicle the history of medical illustration and education in the era of cardiac surgery. Medical illustrators use their talents and expressive ideas to demonstrate procedures and give them life. These methods are (1) hovering technique; (2) hidden anatomy, ghosted views, or transparency; (3) centrally focused perspective; (4) action techniques to give life to the procedure; (5) use of insets to highlight one part of the drawing; (6) human proportionality using hands or known objects to show size; and (7) step-by-step educational process to depict the stages of a procedure. Vivid examples showing these techniques are demonstrated. The result of this observational analysis underscores the importance of the collaboration between clinician and illustrator to accurately describe intricate pathoanatomy, three-dimensional interrelated anatomic detail, and complex operations. While there are few data to measure the impact of the atlas on medical education, it is an undeniable assertion that anatomical and surgical illustrations have helped to educate and train the modern-day surgeon, cardiologist, and related health-care professionals.

George Daicoff was a true pioneer who excelled in patient care, research, teaching, and advocacy; he performed cardiac surgery on thousands. He trained at Mayo Clinic under the tutelage of John Kirklin in 1966. He served on the faculty at University of Florida from 1967 to 1977 and became Chief of Thoracic and Cardiovascular Surgery at University of Florida. He then served as Chief of Cardiovascular Surgery at All Children's Hospital from 1977 to 1998. In 1972, he was one of 13 founding members of the Congenital Heart Surgeons' Society (CHSS), and in 1973, he was one of 10 surgeons to attend the first meeting of CHSS. Dr Daicoff won the 1970 Southern Thoracic Surgical Association President's Award for best scientific paper, and the 1996 Southern Thoracic Surgical Association Osler Abbot Award. In 2015, the Southern Thoracic Surgical Association Congenital Heart Surgery President's Award was named the George Daicoff Award.

Medical students and general surgery residents often do not get exposure to cardiothoracic surgery (CTS) because of a decreased emphasis on CTS rotations during their training. The Southern Thoracic Surgical Association (STSA) began offering the Brooks Scholarship to medical students in 2010 and general surgery residents in 2014 to promote CTS. This study examines the impact of the scholarship.We examined the history of the award and how STSA administers the award. Next we examined the impact of the award by evaluating the number of medical students and residents who are tracked to complete a CTS program. Finally we performed an analysis of the academic output of the awardees who have completed or were tracked to complete a CTS program.The scholarship was developed to honor the memory of past STSA President Dr James W. Brooks. The award is administered through the STSA scholarship committee and provides medical students and residents funds to attend the STSA annual meeting and the opportunity to spend time with a mentor during the meeting. Eighty-eight percent of medical student recipients (21/24) and 100% of general surgery resident recipients (15/15) have completed or are on track to complete a CTS program. The 36 recipients going into CTS have published a total of 823 papers and 9240 articles have cited those papers.The STSA medical student and general surgery Brooks scholarship awards were associated with completing or pursuing a career in CTS. STSA should continue the Brooks scholarship to attract talented medical students and residents to CTS.

The most common long-term complication following arterial switch is supravalvar neopulmonary stenosis. Balloon dilatation has not been effective in treating this problem since the stenosis is often found at multiple levels which will require complex surgical interventions. These include patch augmentation of the supravalvar apparatus, patch augmentation of the distal main and branch pulmonary arteries, and subvalvar muscular resection as needed. The surgical considerations to avoid and treat supravalvar neopulmonary stenosis are: (1) prevention at the time of arterial switch by the use of a pantaloon autologous pericardial patch and (2) application of appropriate surgical interventions at secondary operations for acquired right ventricular outflow tract obstruction. The purpose of this article is to illustrate the optimal methods to achieve these goals.

Background: While epinephrine is the mainstay of therapy during cardiopulmonary resuscitation, it is potentially detrimental to the cerebral vasculature and ineffective in certain populations. This study compares a rescue dose of vasopressin to a rescue dose of epinephrine after ineffective initial doses of epinephrine in diverse models of pediatric in-hospital cardiac arrest. 67 one- to three-month old female swine (10-30kg) in six experimental cohorts from one laboratory received hemodynamic-directed CPR, a resuscitation method where high quality chest compressions are provided and vasopressor administration is titrated to coronary perfusion pressure (CoPP) ³20 mmHg. Vasopressors are given when CoPP is Results: Increases in CoPP and cerebral blood flow (CBF) were greater with vasopressin rescue than epinephrine rescue (CoPP: +8.16 [4.35, 12.06] mmHg vs. +5.43 [1.56, 9.82] mmHg, p=0.022; CBF: +14.58 [-0.05, 38.12] vs. +0.00 [-0.77, 18.24] perfusion units (PFU), p=0.005). Twenty animals (30%) failed to achieve CoPP ³20 mmHg after two doses of epinephrine; 9/20 (45%) non-responders achieved CoPP ³20 mmHg after vasopressin. Among all animals, the increase in CBF was greater with vasopressin (+14.58 [-0.58, 38.12] vs. 0.00 [-0.77, 18.24] PFU, p=0.005).Conclusions: CoPP and CBF rose significantly more after rescue vasopressin than after rescue epinephrine. Importantly, CBF increased after vasopressin rescue, but not after epinephrine rescue. In the 30% that failed to meet CoPP of 20mmHg after two doses of epinephrine, 45% achieved target CoPP with a single rescue vasopressin dose.

There are myriad ethical issues associated with the initiation, maintenance, and termination of ECMO in post-cardiotomy patients. As ECMO becomes more commonly implemented, it is important to consider many of these ethical issues in consenting, counseling, and comforting families who are struggling to grasp the technical, medical and ethical issues that this technology presents.

The treatment of rare and expensive medical conditions confounds care delivery and is one of the defining qualities of pediatric cardiology and congenital heart surgery. Increasing concerns over healthcare resource allocation are challenging the merits of treating more expensive forms of congenital heart disease, and this trend will almost certainly continue. In this chapter, the problems of resource allocation for rare and expensive medical conditions are described from philosophical and economic perspectives. The argument is made that current economic models are limited in the ability to assess the value of treating expensive and rare forms of congenital heart disease. Further, multi-disciplinary approaches are necessary to best determine the merits of treating a patient population such as those with significant congenital heart disease that sometimes requires enormous healthcare resources.

This chapter addresses the ethical issues regarding those situations in which medical and surgical therapy have failed and further action will not alter a patient’s clinical deterioration. The topic of medical futility is discussed at length, including the possible motivations behind its definition and the attempts to codify and define it in modern medicine. That this concept is so difficult to define and is unfortunately persistent in the care of high risk pediatric cardiac surgery and cardiology patients, makes it a very important subject with which congenital heart practitioners should be familiar.

Respect for patient autonomy is an important and indispensable principle in the ethical practice of clinical medicine. Legal tenets recognize the centrality of this principle and the inherent right of patients of sound mind—properly informed—to make their own personal medical decisions. In the course of everyday medical practice however, challenging cases may result in ethical dilemmas for the patient, the physician, and society. Resolution of these dilemmas requires a thorough understanding of the underlying principles that allow the clinician to make informed decisions and to offer considered therapeutic options to the patient. We argue in this chapter that there is also need for a transition of moral competency from understanding principles to attaining virtue in the classic Aristotelian tradition. Achieving moral virtue is based on a lifetime of learning, practicing, and watching how others, who have achieved virtue, act and perform their duties. We further claim that learning moral virtue in medical practice is best realized by incorporating the lessons in daily rounds where frank discussions and considered resolutions can occur under the leadership of senior practitioners who have achieved a semblance of moral excellence.

Clinical ethics consultation is an emerging field whereby an individual or group of individuals with expertise in ethics provides a set of services to clinicians and families to provide guidance about ethical issues that arise during medical treatment. Ethicists may help to resolve disputes when parties disagree about treatment decisions, offer historical context regarding issues that medical providers and families may think are novel, and provide insights into the current state of the issue in the bioethics literature. Multiple methodologies and approaches exist by which an ethics consultant can scrutinize, analyze, and provide recommendations of an ethically acceptable option. In this chapter, we will focus on the role of the ethics consultant, explore various approaches to performing a formal ethics consultation, review cases from the literature, and describe two clinical ethical consultations that involve pediatric patients with congenital heart disease. Finally, we provide an update on the credentialing of ethics consultants and the potential utility of a national ethics database in the future.

This chapter explores the nature of surgical and medical innovation and considers the ethical principles that guide the moral application of innovation to patient populations. Historical milestones are discussed that have resulted in significant innovation in thoracic and cardiovascular surgery that has resulted in the development of the heart-lung machine, open heart surgery, the intensive care unit, myocardial protection strategies as well as countless new operations, modified procedures, and new devices. Historical annotations and careful review of these innovations show that most of these advances were not considered casually and were not impromptu ideas applied haphazardly by surgeons who were seeking acclaim, promotion, or monetary gain. Ethical issues are discussed with regard to regulation of surgical innovation, moral duty, rational discussion, introduction of innovation into, and future considerations. Throughout the chapter are rich historical anecdotes that have framed and highlighted the history of surgical innovation.

Informed Consent in the setting of complex fetal congenital heart disease such as hypoplastic left heart syndrome involves many concerned individuals who include: the mother, the father, the obstetrician, the pediatric cardiologist, the pediatric cardiac surgeon, and many others. It is the duty of the physician to administer and perform informed consent under high risk pre- and postnatal circumstances that require high risk surgical options without which the newborn baby would most certainly die. We explore the intricate roll that physicians play in the informed consent process from prenatal to post-natal circumstances which include making decisions about pregnancy termination, comfort care, and staged palliation. Important considerations such as the importance of the woman’s bodily integrity, autonomy, and the ethics of comfort care are discussed with reference to other complex congenital heart lesions. We conclude that informed consent for HLHS decisions is best practiced by a multi-disciplinary organized approach that will allow comprehensive counseling by multiple care-givers in a timely fashion. Ultimately, the obstetrician/pediatric cardiologist team continue to be the primary physicians that assist with shared decision-making.

Systemic-to-pulmonary shunt failure is a potentially catastrophic complication. We analyzed a large multicenter clinical registry to describe the prevalence and evaluate risk factors.Infants (aged ≤365 days) undergoing shunt operations (systemic artery-to-pulmonary artery or systemic ventricle-to-pulmonary artery) in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) from 2010 to 2015 were included. Multivariable logistic regression was used to evaluate risk factors for in-hospital shunt failure. Model covariates included patient characteristics, preoperative factors, procedural factors including shunt type, and center effects. Centers with more than 15% missing data for key covariates were excluded.Shunt operations were performed in 9,172 infants (118 centers). In-hospital shunt failure occurred in 674 (7.3%). In multivariable analysis, risk factors for in-hospital shunt failure included lower weight at operation (odds ratio [OR], 1.35; p = 0.001), preoperative hypercoagulable state (OR, 2.47; p = 0.031), and the presence of any other STS-CHSD preoperative risk factors (OR, 1.24; p = 0.038). Shunt failure was less likely with a systemic ventricle-to-pulmonary artery shunt than a systemic artery-to-pulmonary artery shunt (OR, 0.65; p = 0.020). Neither cardiopulmonary bypass nor single-ventricle diagnosis was a risk factor for shunt failure. Patients with in-hospital shunt failure had significantly higher rates of operative mortality (31.9% vs 11.1%, p 0.001) and major morbidity (84.4% vs 29.4%, p0.001), and longer median postoperative length of stay among survivors (45 vs 22 days, p0.001).In-hospital shunt failure is common, and associated mortality risk is high. These data highlight at-risk patients and procedural cohorts that warrant expectant surveillance and may benefit from enhanced antithrombotic prophylaxis or other management strategies to reduce shunt failure. These findings may inform planning of future clinical trials.

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient’s economic means, with an emphasis on excellence in teaching, research, and community service.

Biykem Bozkurt, MD, PhD, FACC, FAHA, Chair H. Vernon Anderson, MD, FACC, FAHA Garth N. Graham, MD, FACC Hani Jneid, MD, FACC, FAHA Gail K. Jones, MD David Kao, MD, FAHA Michael Kutcher, MD, FACC Leo Lopez, MD, FACC Gregory Marcus, MD, FACC, FAHA Jennifer Rymer, MD James E. Tcheng, MD

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.Retrospective review of clinical and echocardiographic data of all pediatric patients (age 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).Eighteen children who underwent surgery for severe MR and bileaflet prolapse were identified. All were treated with ring annuloplasty and Alfieri edge-to-edge repair. Median age and weight were 8.2 years (range, 0.4-17.2 years) and 24.9 kg (5.6-63.3 kg), respectively. Median left ventricular end diastolic dimension median z score was 4.9 (2.1-11.9). One patient died (5.6%), and there were no other major complications. Among survivors, 94.4% had mild regurgitation or less, with no stenosis or SAM at median clinical follow-up of 2.4 years (range, 0-13.9 years). Median left ventricular end-diastolic dimension z score regressed to 1.3 (-0.5 to 4.3).In pediatric patients with CTD and severe MR, a simplified approach is associated with intermediate-term competence, absence of SAM or significant stenosis, and regression of left ventricular enlargement.

Endocarditis in adults with congenital heart disease can be traced to intermittent bacteremia owing to dental caries, drug addiction, and chronic infection. Turbulent flow associated with residual congenital lesions such as small ventricular septal defects (VSDs), patent arterial ducts (PAD), and coarctation of the aorta (CoA) cause endovascular damage that can attract and harbor cilia-forming bacteria. In addition, implanted devices such as valve prostheses, vascular grafts, and transcatheter devices can form niduses for bacterial growth and proliferation.

Resternotomy and reoperations to treat the sequelae and residua for congenital heart disease in the adult are associated with multiple challenges of intraoperative hemodynamic management, selective peripheral cannulation, safe reentry, and careful cardiac dissection. Resternotomy is a marker of operative mortality that increases with each successive mediastinal entry. Unwanted cavitary entry during sternal reentry can cause significant hemodynamic instability, especially in patients with prior Fontan operations. Any unexpected decrease in right atrial pressure owing to uncontrolled hemorrhage results in hypovolemic consequences that unfavorably impact the driving force (elevated central venous pressure) that controls the pulmonary circulation and cardiac output. These same sets of circumstances also negatively impact patients who are undergoing ventricular-to-pulmonary conduit replacement, reoperations for tetralogy of Fallot, and tricuspid valve repairs, among others. There are numerous methods of resternotomy. In this chapter, we offer some techniques that have been associated with excellent results and a minimal rate of unwanted cavitary reentry in our collective experience.

Left ventricular outflow tract obstruction in the adult with congenital heart disease is a general term that includes all the obstructive lesions affecting the left ventricle, aortic valve, and the aortic arch. These lesions can be caused by residual defects that were not completely addressed in childhood or by acquired conditions such as hypertrophic cardiomyopathy.