Cardiac malformations are described using several formats of expression, mainly based on three different backgrounds; that is, either from the aspect of embryological development, via a morphological approach, or by means of clinical classifications. We come across occasionally, or probably better to say often, a circumstance in which people misunderstand or are confused with diagnoses of the heart lesions. Common nomenclature for congenital heart diseases is nearly achieved, but not complete yet. This on-going attempt is also being accompanied by the developing coding system. The coding system might not cover all information the heart team needs to share in really practical settings. As long as the way how to describe heart lesions is not perfectly unified, we have to communicate reasonably well realizing that there are several standpoints for diagnoses of cardiac malformations. Behind each word, there are underlying thoughts we should note. Words are derived from concepts, and, at the same time, promote the concepts.

Coronary artery stenosis or occlusion following coronary transfer procedures in congenital heart surgery is a rare but serious complication that often leads to severe heart failure. This complication often needs extracorporeal membrane oxygenation support in the early stage and is associated with sudden death or the need for heart transplantation later after surgery. The complication is particularly important in an arterial switch operation for transposition of the great arteries (TGA), which is performed in newborns and infants with low body weight. Additionally, TGA is often associated with various anatomical coronary abnormalities. Two surgical procedures have been used to manage this complication: surgical redo of coronary ostial anastomosis, often with autologous tissue patch enlargement (SOAP) and pediatric coronary artery bypass surgery using the internal thoracic artery (PCABS-ITA). Both methods have relative advantages and disadvantages but early surgical survival results are equivalent. Based on various database analyses, I currently consider SOAP as I(C) and PCABS-ITA as IIa(C) for a rescue operation in which coronary obstruction is due to technical errors or mechanical compression, kinking, and/or stretching. For late coronary complications in which fibroproliferative obstruction is the main cause, I recommend PCABS-ITA as I(C) and SOAP as IIa(C). In addition, tight stenosis (>90%) or total obstruction extending into the bifurcation of the left main trunk favors PCABS-ITA, whereas localized left main stenosis of a less severe degree favors SOAP. Careful follow-up and long-term results are important. Because coronary obstruction is a serious but rare complication, analysis of long-term data is essential.

The Ross procedure is an excellent option for aortic valve replacement. This double-valve replacement technique is particularly well-suited for neonates, infants, children and adolescents, offering several advantages, including excellent long-term survival, low thrombogenicity, growth potential, and excellent hemodynamic performance. However, the Ross operation proposes disadvantages, such as surgical technical challenges and the future risk of two valve disease from both autograft failure and the necessity for reintervention on the pulmonary allograft. This review aims to examine the contemporary outcomes of the Ross operation in children in our institution over 30 years. Also, insights from other clinical studies with single institutional experiences, multicenter registry data, and expert opinions are synthesized to provide a comprehensive overview of the current state of the Ross procedure. The data were stratified by age to provide more insights into technical, physiological, and outcome differences between infants, children, and adolescents. Although the excellent long-term outcomes after the Ross procedure have been recognized in the young adult population, it remains challenging, especially in infants, due to a higher early mortality rate and the unavoidable need for reintervention for the pulmonary allograft. Continued research, technological innovations, and collaborative efforts among healthcare providers are essential to refine this technique further, ensuring its widespread applicability and improving the quality of life for pediatric patients with aortic valve disorders.

High-resolution three-dimensional mapping has rarely been used in patients with multiple accessory pathways. We report a 5-year-old girl with a diagnosis of paroxysmal supraventricular tachycardia who was referred to our pediatric unit for catheter ablation. Electrophysiological study revealed a left accessory pathway and orthodromic atrioventricular reciprocating tachycardia (AVRT). After trans-septal puncture, we used the ORION™ high-density catheter and the RHYTHMIA™ Mapping System to map an activation pattern of the left atrium during right ventricular pacing. Activation mapping revealed that the earliest site of atrial activation occurred in the left lateral portion of the left atrium, and, in addition, anterolateral activation sometimes occurred as early as left lateral activation. After the first radiofrequency catheter ablation on the left lateral atrium, narrow QRS complex tachycardia persisted, and the earliest atrial activation occurred in the anterolateral portion. Two AVRTs via separate accessory pathways were diagnosed; those two accessory pathways were 16.7 mm apart. High-resolution activation mapping showed two breakthrough activations via the mitral annulus. The propagated waves collided at the distal portion of the left atrium through the mitral annulus. This case showed that ultra–high-resolution mapping is safe and useful for delineating multiple accessory pathways, even in pediatric patients.

We report a case of 38-year-old female with congenitally corrected transposition of the great arteries and ventricular septum defect. She had undergone physiological repair 28 years ago and closure of residual ventricular septum defect 19 years previously. She was listed for heart transplantation one year ago due to severe systemic right ventricular dysfunction, readmitted two months back due to worsening heart failure, and became inotropic support dependent. Implantation of a ventricular assist device was indicated as a bridge to heart transplant. A standard implantation method from the apex of the heart to the systemic right ventricle was challenging due to mesocardia and longitudinal configuration of the right ventricle. Therefore, under transesophageal echocardiogram guidance, the 3rd generation ventricular assist device was vertically placed from the diaphragmatic surface towards the aortic valve. The postoperative course was uneventful, and the patient was waiting for heart transplantation as an outpatient.

Thrombus formation within the left atrial appendage is extremely rare in neonates. For intracardiac thrombus, anticoagulation therapy is the most common option of treatment. In high-risk cases of systemic embolism, however, surgical resection should be considered. We experienced a neonatal case with a left atrial appendage thrombus related to transient left ventricular dysfunction without congenital heart malformation; the definitive etiology of cardiac dysfunction remained unknown. Daily echocardiography revealed that mobility of the thrombus became prominent as the left ventricular systolic function improved. Surgical thrombectomy was performed due to an imminent concern of its embolic risk. We herein report details of the highly suggestive clinical course in our patient.