Journal of Pediatric Cardiology and Cardiac Surgery

One day, George sat in a lecture room together with his peers, awaiting the arrival of a professor of cardiac morphology. The professor was famous for a systematic approach to analyzing the structure of heart defects. George had only a limited knowledge of congenital cardiac malformations. He attempted to read a textbook on cardiac embryology three years ago when he was a medical student. He remembered that he was not able to complete any of the chapters. Each time he started from the beginning of a chapter to make sure that he could understand perfectly, the book always turned out to be a nice pillow on his desk within 20 minutes. To him, pictures were just like abstract artistic shapes, their titles being labelled in Latin. On the basis of this discouraging memory, he was hoping that today’s lecture would not be a further setback.

“Good morning, ladies and gentlemen.” The professor began his talk brightly. With his loud and clear voice, I could not sleep through his lecture, George thought. The professor explained very precisely the architecture of the normal heart. Not too fast, not too detailed, not too specialized. His interactive presentation created a nice atmosphere. Subsequently, he moved on to one example. He showed how to analyze the abnormally structured heart step by step. At the end, the descriptive list on the specimen became extensive (Table 1a). George realized that he needs some verbal tools to find out features of a structure in detail. To recognize an object is rather different from just to see it; the former needs active participation of mind, he thought. When George went back home, however, he was not entirely sure whether he could recall all the key points the professor demonstrated during the day and whether he could apply the principle to each case he might have at the time of examination. Therefore, he decided to buy a book written by the professor. He actually read the book with great interest. The way of thinking termed “sequential segmental analysis” seemed to be a good conceptual tool to him. He felt that everything would become clear-cut when he used such a systematic and scientific approach.

One year later, George had an opportunity to attend a short course on congenital heart disease abroad. He was hoping to augment his knowledge in addition to the morphologic approach he had learned. He did not suspect that topics would be discussed without using the “sequential segmental analysis” taught by the professor. Therefore, he was quite shocked to find that he could not understand completely what people were talking about. “Bilateral conus,” “endocardial cushion,” “Malposition of the great arteries,” “SDL,” “polysplenia,” and so on. He felt he was on another planet. He wondered if everything might have changed for diagnoses of cardiac malformations during the past 12 months. After finishing the afternoon session of the first day, he asked one of the young participants in a friendly manner. “I did not understand very well the terminology the lecturers used today. Where were they from? What does l-loop mean?” “Well, you need to study more about the background of heart defects. Congenital malformations cannot be understood without knowledge of embryology, you see,” the young man replied bluntly. “Embryology!” George exclaimed in his mind. “That’s what I could not cope with in the past,” a feeling of despair took the shine out of his eyes. After supper, he was still reflecting on what he had listened to during the day. “Am I hopeless? Is this field beyond my ability? But, probably, I could have understood some of the contents with knowledge of “sequential segmental analysis,” couldn’t I?” That night, George had a nightmare in which he was confronted with a menu written in Arabic characters and unable to order anything for dinner.

Next day, the morning session was all right; imaging modalities were explained on relatively simple diseases. At lunch time, a senior lecturer sat next to George and gently asked, “How is the course going? Everything clear?” George replied frankly, “I was confused yesterday. I do not have enough knowledge of embryology. I could not understand the terminology and underlying concepts very well. I previously learned the basis of sequential segmental analysis. Is that completely useless?” The gentleman smiled and quietly said, “Embryology is an important aspect in order to understand congenital diseases. Providing background knowledge allows us to interpret and explain features of the malformed hearts. Having said that, it is not the only approach we have. Purely morphological description can, in practice, show us detailed architecture, whereas other designated stereotypes are also clinically relevant. I have to admit there are conflicts within the nomenclatures because we intend to establish academic terms as precisely and as specifically as possible. Still, the essence of these scientific dogmas exists commonly in the ultimate understanding of congenital heart diseases. By the way, did you know that the systematic approach to cardiac segments and their sequence was derived from an embryologic context initially?” “Interesting,” said George. “In other words, the same condition is described in different languages according to academic standpoints, isn’t it?” “That’s correct.” “Diagnoses made on a common matrix would have been easier for a beginner like me to understand. How do you describe this sort of malformation for example?” George illustrated an example that was nicely explained before on the basis of morphology. The gentleman explained his thought and its background patiently (Table 1b). George thanked him greatly for the fruitful personal tuition.

Soon after starting specialist training in cardiology a couple of years later, George came across a patient with malformations similar to that memorable case. When George began to present the morphologic diagnoses to a surgeon, he impatiently cut George’s words short. “TA/TGA and CoA, isn’t it? (Table 1c). How restrictive is the VSD? Is the aortic valve bicuspid? The aortic arch hypoplastic? Body weight? On prostaglandin? Lactate level? ……” George was flustered. The surgeon immediately taught George what to say, how, and why. “The expression you attempted initially should be precise, but it is far too long-winded for a busy surgeon like me,” he added impishly. “If you presented the case at a scientific meeting in your manner, you would use half of your limited presentation time just for the morphologic diagnoses!” This time, George was not frustrated. “Another stance,” he muttered to himself. Beyond accuracy, well-summarized diagnosis and clinical key points were needed. He also learned that attention should be paid to presence or absence of potentially possible impediments.

George has expanded his knowledge on many fronts. He experienced cardiology, particularly for congenital heart disease, at several institutions all over the world. He came to realize that, not only nomenclature of diagnoses, but also background concepts and practical skills of treatments were really varied. He felt people were occasionally on quite different wavelengths. “Sometime, we might be able to build up a consensus in a true sense. At present, at least, we need to prepare and to widen the scope for deeper knowledge so as to translate each opinion into a meaningful and non-misleading one,” he calmly said to his new trainee.

It should be useful to have a definitive dictionary of terminology in congenital heart diseases. As is often the case with dictionaries, for example between two languages, different grammars and background cultures make it very difficult to give exacting one-to-one correspondence in everything. No dictionary guarantees perfect translation on its own. Nonetheless, some examples are provided by listing similar, or comparable, expressions in comparison (Table 2).

To classify stereotypes or subtypes of a spectrum of malformations, headings were given consisting of abbreviations and figures in the past (Table 3). Some people continue to use such headings with a sense of pride that they have studied meticulously the past literature, while others avoid using insipid typification with the explanation that they are confused with the digits and alphabets. If a classification is not well adopted, communication and understanding of real malformations are difficult.

Any descriptions in the literature should aim to be as accurate, as suggestive, or as practical as possible. At the same time, each one has its downside.

Detailed morphological diagnoses are occasionally extremely long. Of course, to be precise is of utmost importance. But, it is a reality that some people feel reluctant to describe every aspect in long-hand, especially in this era of text-messaging. This is more often the case in those whose first language is not English.

On the other hand, embryology requires considerable effort for understanding. We need to accumulate bulky knowledge. Precise details of normal development are still being elucidated, let alone controversies in abnormal development. Obviously, brief embryologic terms imply background insight into development of the heart, some of which may be presumptive. Because of various levels of knowledge, communication between discussants and the audience is not always smooth.

Truncated expressions also need some background knowledge of the literature, and, more importantly, they are occasionally incomplete, confusing, or misleading. Such shorthand could be used within a clinical team, but not recommended in academic circumstances.

As long as no unified way is established, at least at present, we have to communicate reasonably well realizing that there are several standpoints for diagnoses of cardiac malformations. Common nomenclature for congenital heart diseases is being negotiated^23–26) or nearly established even, but not completely yet. This on-going attempt is also being accompanied by the developing coding system.^27–30) Behind each word, there are underlying thoughts we should note. Words are derived from concepts, and, at the same time, promote the concepts.