To Our Primary Care Physicians

It is currently estimated that there are nearly 600,000 adults in Japan with congenital heart disease (ACHD), and with that number reportedly increasing by 10,000 each year, I imagine that physicians of all specialties have opportunities to examine ACHD patients with some regularity. When consulting with young working adults or students about their daily lives, you may find yourself asking questions unique to rare diseases, such as, “Is it really safe for this patient to continue coming for regular checkups as is?” “Could their condition suddenly worsen if they don’t undergo additional treatment at some point?” or “What kinds of specialized tests are available, and where can they be performed?” In recent years, many ACHD patients—even before reaching the age of coming-of-age ceremonies—have been experiencing anxiety about school life, further education, and employment as early as adolescence. Consequently, it has become increasingly common to consider their care from an AYA (adolescent and young adult) perspective at an earlier stage, and I believe there are many attending physicians who are managing these patients with a long-term view of their future.
We established this online consultation service to address the minor questions and concerns of such physicians, serving as a resource that allows them to provide care with peace of mind while supporting their patients. As is well known, patients with adult congenital heart disease are born with complex anatomical conditions and may undergo multiple corrective surgeries; over the course of decades, problems with cardiac function, valves, and blood vessels can accumulate. At the same time, the history of cardiac surgery is still relatively short, and there is a lack of sufficient clinical evidence; therefore, it is no exaggeration to say that each patient faces unique challenges.
Dr. Itatani is a cardiac surgeon who has been at the forefront of ACHD surgery in Japan from the very beginning. In the past, he established Adult Congenital Heart Disease Centers in several government-designated cities and has dedicated himself to addressing both surgical and hemodynamic issues. He has now joined the Department of Cardiovascular Surgery at Juntendo University to build a larger and more inclusive patient care network. At this consultation office, we are able to provide advice from a specialized surgical perspective on issues related to hemodynamics and cardiac function in ACHD, addressing questions such as: “What types of specialized tests are appropriate for which conditions and at what stage?” or “If additional treatment is needed in the future, when would it be appropriate to consider it, and how might it affect work and daily life?”

Reoperation for Congenital Heart Disease in Adults

In cases of congenital heart disease in adults, heart valves or blood vessels repaired during childhood may require re-treatment, or patients may develop arrhythmias or heart failure as they grow, necessitating treatment. These surgical procedures often involve repeat open-heart surgery and are considered particularly challenging and high-risk.
Such surgical interventions are not limited to patients in life-threatening emergencies; they are also frequently necessary for patients who experience fatigue or palpitations in their daily lives. In particular, many adult patients with congenital heart disease are attending school or working and leading active social lives; it is therefore crucial for them to undergo surgery at the appropriate time to ensure they can withstand the cardiac strain associated with work, exercise, pregnancy, and childbirth.
However, because surgical procedures for congenital heart disease in adults are extremely complex, the reality is that even in developed countries, including Japan, there are very few hospitals capable of performing such surgeries. Dr. Itatani is a cardiac surgeon specializing in these conditions. With extensive surgical experience, he has treated a wide range of pathologies involving complex anatomy. In the past, he has performed the following advanced surgical procedures. Through this online consultation service, he is available to advise patients who are unsure whether to seek a second opinion or who are considering when and where to undergo surgery. Below is a list of the surgical procedures Dr. Itatani has specialized in to date.

• Pulmonary valve replacement in adulthood following repair of the pulmonary valve (e.g., in Tetralogy of Fallot), as well as procedures performed concurrently with mitral or tricuspid valve repair and arrhythmia surgery
• Revision surgery following repair using an artificial graft with a right ventricular outflow tract valve (Rastelli procedure) for conditions such as Tetralogy of Fallot
• Revision surgery on the aortic root following congenital heart disease surgery (autologous valve preservation, prosthetic valve replacement)
• Valvuloplasty and valve replacement for congenital atrioventricular valves
• Reoperative procedures for hemodynamic instability or arrhythmias following single-ventricle repair (post-Fontan surgery)
• Surgery for biventricular pacing due to right ventricular failure or atrioventricular regurgitation
• Valvuloplasty and valve replacement for tricuspid valve diseases, such as Epstein’s anomaly
• Medical therapy and combined surgical treatment for shunt defects (atrial septal defect, ventricular septal defect, atrioventricular septal defect, anomalous pulmonary venous return, etc.) complicated by pulmonary hypertension or valvular heart disease
• Aortic arch surgery for patent ductus arteriosus and aortic coarctation
• Coronary artery repair and coronary artery bypass surgery for congenital coronary artery disease

On Cutting-Edge Research in Blood Flow Analysis

Such complex surgeries cannot be easily performed based solely on accumulated surgical experience; in fact, it is generally acknowledged that, when it comes to congenital heart disease in adults, there is insufficient accumulated experience and data, and many aspects remain unexplained. Dr. Itatani has pioneered the development of a method—known as blood flow analysis—to visualize and simulate blood flow within the heart and blood vessels. While blood flow analysis is a cutting-edge field of medical research, when combined with appropriate clinical care, it enables a comprehensive assessment of each patient’s cardiac issues and allows for meticulous treatment planning.

This information plays a crucial role in understanding the cardiac function and hemodynamic issues associated with complex congenital heart defects. Even without surgery, I believe that undergoing a 4D imaging examination—which provides a clear, detailed understanding of cardiac function and hemodynamics in congenital heart defects—and confirming that there are no problems with the heart or blood vessels will allow you to live your life with peace of mind. You can consult with us through this online consultation service to discuss whether you should undergo such an examination. Additionally, if requested, we will share the analysis results for patients referred to us separately from our standard medical information provision.