An early model of heart formation suggested that the heart is sequentially formed through the anatomical compartmentalization of the cardiac primordium along the anteroposterior axis, from the outflow tract to the atrium. However, this model has drastically changed since the proposal 20 years ago of the secondary heart field, where cardiac progenitor cells exist on the medial side of the cardiac primordia. Recently, transcriptome analyses of single cells have shown that the fate of each anatomically segmented region may be determined in a different spatial and temporal order depending on the timing of mesoderm formation, thereby prompting further refinement of the model. Cell fate is determined by mesodermal cell migration to the anterior part of the embryo to form the cardiac primordium, including designated cardiac regions, along the mediolateral axis. The heart is formed when a portion of the cardiac primordium changes morphologically and remains as a pool of cardiac progenitor cells supplying the inflow and outflow tracts. The behavior of cells from the progenitor pool during heart formation is now better understood. This review evaluates recent findings and provides an updated model of heart formation.

Fetal echocardiography is an essential test for the diagnosis of congenital heart disease and perinatal management strategies. Because of the constantly changing position of the fetus, different echocardiography techniques are required to obtain the basic cross- sectional images necessary for diagnosis. Since fetal echocardiography is mainly based on horizontal cross- sectional scanning to evaluate the heart and its great vessels, learning the techniques for obtaining a “clear four-chamber view” and “easy-to-observe four-chamber view” is recommended to improve fetal echocardiography. By applying these techniques, sagittal and short-axis cardiac sections can be visualized, and echocardiography for complex fetal cardiac malformations can be performed with confidence. In this article, we explain the concepts in fetal echocardiography and the techniques for obtaining clear cross- sectional images.

Background: The prevention of coronary artery aneurysm is important in the treatment of Kawasaki disease (KD). Thus, the ability to predict the risk of coronary dilation before initiating therapy would be very useful. In this study, patients suspected of KD underwent echocardiography and were evaluated for specific findings. We observed for specific changes that occurred in the course of KD, including when they appeared and their association with coronary dilation (Z ≥2.5).

Methods: We evaluated 169 patients diagnosed with KD. We analyzed the associations of coronary dilation with specific echocardiogram findings, including tricuspid regurgitation, pulmonary regurgitation, mitral regurgitation, aortic regurgitation (AR), pericardial effusion (PE), left ventricular end-diastolic diameter, and left ventricular ejection fraction, during hospitalization using univariate and multivariate analyses.

Results: Univariate and multivariate analyses showed that AR, PE, and left ventricular dilation were significantly associated with coronary dilation. Specifically, AR preceded coronary dilation, and its positive predictive value for coronary dilation was 30％, sensitivity was 42％, and specificity was 76％.

Conclusions: AR, PE, and left ventricular dilation were associated with coronary dilation, with AR preceding its occurrence. Therefore, patients with KD with AR early in the diagnosis require careful follow-up. Furthermore, prompt intravenous immunoglobulin therapy should be considered.

Background: The number of adult patients with congenital heart disease (CHD) who get married, become pregnant, and give birth is increasing with the improvement in their prognosis. We examined the hope and cognition of the marriages, pregnancies, and childbirth of women with CHD.

Methods: A questionnaire-based survey was conducted on 307 adult women with CHD (aged 20–49 years) regarding their heart diseases, marriages, pregnancies, and childbirth. We divided the subjects into two groups according to whether they had or had not acquired the first grade of the physical disability certificate.

Results: Responses were obtained from 89 participants (valid response rate: 29.0％), including 53 acquirers and 36 nonacquirers. The number of married people was significantly more frequent in the acquirer than in the nonacquirer group. In both groups, 80％ were aware of the “Burden on the heart due to pregnancy” and “Necessity of prepregnancy examinations.” The acquirers were highly aware of “Influences of heart disease medications on the fetus” and “Inheritance of congenital heart disease by the fetus.”

Conclusions: Both groups reported high aspirations for marriage, pregnancy, and childbirth. They need to be supported and educated continuously for a safe pregnancy and childbirth by a multidisciplinary team.

Background: Infants with congenital heart disease living at home may have unscheduled hospitalizations. Efforts to moderate unplanned hospitalizations require clarifying the causation of such incidents; therefore, we conducted a study to identify the related etiological factors along with the total number of children affected.

Methods: The medical records of children with congenital heart disease in infancy who had been hospitalized at hospital A during a period of 5 years were collected and analyzed.

Results: Unplanned hospitalization was required for 35.4％ of children with congenital heart disease in infancy. Most of these hospitalized patients were aged ＜1 year (31.7％), and many of them had chromosomal abnormalities. The main causes were respiratory complications such as pneumonia and bronchitis as well as worsening cardiorespiratory status, including heart failure, low SpO₂, cyanosis, and hypoxemia. Home oxygen therapy was used in 50.3％, home ventilatory therapy in 7.9％, and home tube-feeding therapy in 26.0％.

Conclusions: Unplanned hospitalization of children with congenital heart disease was most common in children less than 1 year of age. It was affected by the underdevelopment and vulnerability of respiratory and circulatory functions. Minimizing unplanned hospitalizations will require seamless support from both medical professionals in the institution and nursing staff in the home.

Background: The purpose of this study was to look at the current state of the adult patients with tetralogy of Fallot, the most common cyanotic congenital heart disease, to encourage social independence and healthy lifestyles.

Methods: On 186 patients, a questionnaire survey (understanding and anxiety about their heart diseases, treatments, social independence, and lifestyles) was administered. These data were also compared between patients with and without physical disability certification (a certified group and a noncertified group). Clinical data were extracted from the medical records.

Results: After excluding the cases without meeting the inclusion criteria, 112 patients (41 males, mean age 28 years) were studied. Eighty-three percent of 93 patients after excluding 19 students, were employed (66％ full-time employee), half of them lived with their parents, and 71％ were concerned about their heart diseases. In terms of lifestyle, 28％ were dissatisfied with the quality of their sleep. The noncertified group (n=59) was assigned more professional tasks, whereas the certified group (n=53) was assigned more office duties. The certified group had more regular outpatient clinic visits and dental consultations, but also had a greater experience to drink alcohol and take a sleeping pill.

Conclusion: The study patients had a relatively good job rate and a high level of social independence, despite having a variety of anxiety disorders. It was suggested that some supports for anxiety and sleep disorder issues be implemented especially in the certified group.

Late aortic aneurysm is a rare complication of subclavian flap aortoplasty (SCF) for coarctation of the aorta (CoA). Here, we describe the cases of two adult patients with late aortic aneurysm after SCF. The first patient was a 26-year-old man who underwent SCF for a simple CoA at 3 years old. He had a fusiform type of aortic aneurysm located at the site of SCF. Aortic graft replacement was successfully performed under deep hypothermic circulatory arrest using the retrograde cerebral perfusion technique through a median sternotomy. The second patient was a 34-year-old woman who underwent SCF for a simple CoA using SCF at 6 years old. She had a saccular type of aortic aneurysm in the distal lesser curvature wall of the SCF. Aortic graft replacement was successfully performed using the partial bypass technique through a left thoracotomy. The patients were stable without late complications postoperatively after 14 and 13 years, respectively. Long-term follow-up is required for late aortic aneurysms after SCF.