Background: A carotid artery approach is advantageous for cardiac catheterization in infants and children. Conventionally, the surgical cutdown access method is popular; however, recent advancements in medical device technology have led to the development of the percutaneous carotid access method. This study aimed to evaluate the advantages and safety of percutaneous carotid access using 3-French sheath compared with those of surgical cutdown access.

Methods: We retrospectively reviewed data of 17 children who underwent cardiac catheterization via the carotid access approach between January 2003 and April 2021. Patients were divided into two groups: those who underwent surgical cutdown (Cutdown group, 2003–2013; n=10) and those who underwent percutaneous carotid access (Puncture group, 2014–2021; n=7).

Results: The median ages at the time of procedure were 2 months and 1 month in the Cutdown and Puncture groups, respectively (p=0.13). In the Cutdown group, three, four, and three patients required sheath sizes 3-French, 4-French, and 5-French or larger, respectively; on the other hand, a 3-French sheath was used in all patients in the Puncture group. The median time to vascular access and that from sheath removal to exit the room were 33 min and 36 min in the Cutdown group, and 3 min and 25 min in the Puncture group, respectively (p=0.01). No patients exhibited complications related to vascular access.

Conclusions: The percutaneous carotid access approach with a 3-French sheath in infants has the potential to reduce procedure time and to provide a comparable or safer, less invasive catheterization than with the conventional method.

Background: Since most patients with 22q11.2 deletion syndrome (22q11DS) have congenital heart defects (CHD), they are usually followed by pediatric cardiologists. Throughout their life, about two-thirds of patients with 22q11DS suffer from developmental and psychiatric disorders. In this setting, pediatric cardiologists may be the first health care providers who need to identify psychiatric problems, but the actual state of psychiatric care in routine cardiology check-up remains unknown.

Patients and Methods: We retrospectively reviewed the numbers of 22q11DS patients with both psychiatric disorders and CHD in 12 collaborating facilities specialized in CHD in Japan (primary survey). In two elective hospitals where a comprehensive healthcare program for patients with 22q11DS has been provided, further surveys were carried out to collect detailed information regarding the clinical profile of psychiatric disorders and CHD (secondary survey).

Results: Two hundred and twenty-two patients with 22q11DS and CHD were reported to have psychiatric disorders. While thirty-nine out of 222 patients (18%) received specialized care by psychiatrists, the rest (82%) were followed by pediatric cardiologists in the primary survey. Twenty-seven patients were included in the secondary survey, and 26 patients (96%) were treated for psychiatric problems by psychiatrists. The median age at onset of psychiatric disorders was 24 years, and 59% of them were diagnosed with schizophrenia.

Conclusions: In this nationwide survey, most of pediatric cardiologists did not consult experts about psychiatric problems of 22q11DS. Aiming to improve prognosis, early collaboration with psychiatrists is necessary for pediatric cardiologists facing psychiatric disorders, especially schizophrenia.

We herein report a recurrent case of Kawasaki disease (KD) complicated by giant coronary aneurysms that developed only fever and slight conjunctival injection. A 3-year-old boy developed a cough and fever for one day and was referred to us because of a strong inflammatory reaction. He had a history of complete KD previously, and was treated with intravenous immunoglobulin therapy (IVIG) 7 months ago with no coronary arterial lesion afterward. However, this time, he had a cough and chest X-ray showed consolidation; therefore, we suspected bacterial pneumoniae and started antibiotic therapy. Since this failed to reduce his fever, we administered another antibiotic drug, which was also ineffective. Echocardiography was performed on day 7 from onset and revealed bilateral coronary arterial aneurysms, the appearance of which resembled a string of beads. Based on this finding, he was diagnosed with incomplete KD and IVIG, cyclosporine A, and aspirin were administered. Fever improved 12 hours later, but recurred at 36 hours. Due to the possibility of the further expansion of coronary arterial aneurysms, he was transferred to another hospital for plasma exchange.

Patients with recurrent KD are known to be at risk of coronary arterial lesions. However, since the present case only exhibited 2 main manifestations, making an accurate diagnosis was not straightforward. Therefore, the recurrence of KD needs to be considered in children with fever and a history of KD.

A 30-year-old woman with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) treated with Takeuchi repair when she was one-month-old was referred to the obstetric department of our hospital for the management of her first pregnancy. She had a surgical operation for severe pulmonary regurgitation and tricuspid regurgitation one year before the pregnancy. The preoperative study showed a deterioration of right ventricular (RV) function, while presenting good performance of the left ventricle (LV). The cardiac function was found fully recovered through a postoperative study. A multidisciplinary medical team made close observations from the 3rd trimester to after the delivery. A successful vaginal delivery occurred at 39 gestational weeks with a completely healthy baby without any maternal cardiac events. The serum brain natriuretic peptide level did not elevate significantly during the pregnancy. The echocardiography showed that the LV ejection fraction, the LV internal dimension in diastole, and the global longitudinal strain (GLS) of LV remained in normal ranges. No significant valvar insufficiencies or stenoses were found. The GLS of the free wall of RV changed from −19.7% to −10.7% during the delivery and recovered to −23.8% one month after the delivery. Analogously, the fractional area change worsened from 58.3% to 25.4% and returned up to 57.1%. The tolerance of the cardiac load in pregnancy and delivery was ensured by the excellent postoperative course of ALCAPA with Takeuchi repair as well as the successful pulmonary and tricuspid valve reoperation in adulthood with fine assessment of the RV function.

An autograft aneurysm with possible autograft regurgitation is one of the well-known complications late after the Ross procedure, and various modifications to avoid autograft aneurysm and autograft regurgitation had been reported. We present a middle-aged gentleman with distal autograft aneurysm with ascending aortic aneurysm despite the aortic root fixation by Dacron felt at the time of the Ross procedure 20 years ago. The valve-sparing root replacement by David technique was planned, but converted to Yacoub technique during the operation due to severe calcification of the fixation material with difficulty of aortic root dissection. The Ross procedure concomitant with autograft root fixation technique was able to prevent the autograft annulus dilatation; however, unable to prevent autograft sinus of Valsalva aneurysm. Yacoub technique was an excellent option to proceed with valve-sparing root replacement operation for an aneurysmal autograft with concomitant root fixation.

There are few reports of myocardial infarction in childhood and adulthood caused by a thrombotic obstruction due to a coronary aneurysm (CA), which is considered to be a sequela of Kawasaki disease, in a case in which the history of Kawasaki disease is unknown. Here, we report a case of a 6-year-old boy with no history of Kawasaki disease who developed acute myocardial infarction (AMI) caused by thrombotic occlusion by a right coronary artery (RCA) giant aneurysm. The patient experienced anterior chest pain and vomiting, and was transferred to our hospital because of lethargy. Acute myocarditis was suspected based on an abnormal Q wave and ST depression on an electrocardiogram, and a decrease in left ventricular ejection fraction on echocardiography. Echocardiography revealed a giant RCA aneurysm and a thrombus-like low-intensity region within the aneurysm. Coronary computed tomographic angiography also showed a giant CA and occlusion at the same site. AMI due to thrombotic obstruction by the RCA aneurysm was suspected. Anti-heart failure treatment and anticoagulant therapy were started, and led to recovery of cardiac function. Coronary angiography performed 3 months after onset revealed complete occlusion of the RCA. Collateral circulation from the left coronary artery was confirmed and recommunication between the right coronary arteries was partially observed. In a case of ischemic chest pain and impairment of cardiac function in childhood, careful examination and appropriate treatment are required regardless of the history of Kawasaki disease, based on the possibility of myocardial infarction due to occlusion by a CA.

Short QT syndrome (SQTS) is an inheritable cardiac electrical disease presenting both atrial and ventricular arrhythmias associated with abnormally short QT intervals on electrocardiograms (ECGs). SQTS is mainly associated with mutations of the genes encoding three different cardiac potassium channels. Among them, type 2 SQTS (SQT2) can be caused by gain-of-function of KCNQ1, resulting in accelerating ventricular repolarization. Several studies reported that patients with SQT2 bearing the KCNQ1 c.421G>A: p.V141M variant occasionally suffered from atrial fibrillation or bradycardia but rarely developed ventricular arrhythmias, and thus the variant has been considered as benign. However, when we observed an SQT2 family with the KCNQ1 p.V141M variant, one of the family members had developed ventricular fibrillation. The proband was referred to our hospital due to severe bradycardia (atrial standstill) and short QT intervals at the age of two and received a pacemaker implantation (PMI) using epicardial ventricular leads. The proband’s father also underwent a PMI at the age of 20 due to sick sinus syndrome. Genetic testing, performed to investigate familial bradycardia, identified a heterozygous KCNQ1 p.V141M variant in the proband and the father. Since the PMI, the father had been stable until at the age of 42 when he had a syncope due to ventricular fibrillation (VF). The VF was successfully terminated by an automated external defibrillator, and an implantable cardioverter-defibrillator was implanted. This is the first reported case of a patient with SQT2 bearing the KCNQ1 p.V141M variant showing lethal ventricular arrhythmia.

We present a case of massive pulmonary arterial thrombosis that occurred in an infant, during the early postoperative period after complete surgical repair of a severe form of Tetralogy of Fallot. The patient required emergent circulatory support with veno-arterial extracorporeal membrane oxygenation immediately followed by percutaneous thrombus fragmentation and local thrombolysis, which enabled partial thrombus removal and hemodynamic stabilization. Subsequently, the patient was scheduled for urgent surgical thrombectomy and central pulmonary arterial enlargement plasty, with successive circulatory and clinical improvement. The etiology of this complication was suspected to be multifactorial; multiple surgeries, systemic inflammatory state, hypoplastic pulmonary arteries, and moderate prothrombotic state. A multidisciplinary collaboration between intensivists, cardiologists, and cardiac surgeons and a rationale and step-by-step clinical approach were paramount to face this rare life-threatening complication.