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Background Rifampicin is a strong inducer of the hepatic cytochrome P450 (CYP) family and is known to interact with many clinical drugs. However, to our knowledge, no case of worsening heart failure (HF) due to the interaction between rifampicin and HF drugs has been reported. Case summary A 32-year-old female, who had undergone intracardiac repair for an incomplete atrioventricular septal defect with dextrocardia and prosthetic valve replacements for right and left atrioventricular valve regurgitation, presented as an outpatient. Her medications included tolvaptan 15 mg and warfarin 1.25 mg. She had a slight fever and Osler nodes at her fingers. Blood culture bottles grew methicillin-resistant Staphylococcus epidermidis , and several vegetations were observed on the right atrioventricular mechanical valve with a transoesophageal echocardiogram. She was diagnosed with prosthetic valve endocarditis and treated with antibiotic agents including rifampicin. After a week, she developed systemic oedema and had a marked decrease in prothrombin time–international normalized ratio (PT-INR). Rifampicin was promptly discontinued due to a strong suspicion of a drug–drug interaction. Consequently, both her congestion and the PT-INR stabilized, and she was discharged after 8 weeks of antibiotic treatment. Discussion The introduction of rifampicin induces CYP family members such as CYP3A4 and CYP2C9. Warfarin is metabolized by CYP2C9 and tolvaptan is also metabolized by CYP3A4, resulting in a notable reduction of their blood levels when co-administered with rifampicin. The clinical challenges arising from interactions between HF drugs and rifampicin can be categorized into two main groups: worsening HF and thrombotic complications. Clinicians should remain vigilant and informed about these potential issues. [ABSTRACT FROM AUTHOR]

The management of systemic artery aneurysms secondary to Kawasaki disease (KD) in adults remains a therapeutic challenge. KD guidelines recommend the use of anticoagulation therapy with warfarin in addition to antiplatelet therapy when a giant coronary aneurysm or a history of thrombosis is documented. However, long-term use of warfarin presents several concerns. This case reports acute thrombotic occlusion due to the giant arterial aneurysm in an adult KD. A surgical resection of the aneurysm was performed because of recurrent thrombotic events, despite anticoagulant therapy with warfarin. Pathological examinations revealed a layered thrombus with inflammation in the aneurysm and Factor Xa expression mainly in newly formed thrombus. This study provides an insight into the anticoagulation therapy for cardiovascular sequelae after KD. < Learning objective: This study, along with pathological evidence, illustrates that Factor Xa might contribute to thrombotic events after Kawasaki disease.> [ABSTRACT FROM AUTHOR]

We report the first case of transcatheter perimembranous ventricular septal defect (pmVSD) closure in Japan where none of existing devices for VSD closure has been approved. The pmVSD was successfully closed with first generation Amplatzer® duct occluder (ADO-I; St Jude Medical, St Paul, MN, USA). The procedure was performed under general anesthesia with transesophageal echocardiographic and fluoroscopic guidance. The left ventricular volume overload after the procedure was remarkably improved and no major complications occurred. ADO-I can be a safe and effective option for transcatheter pmVSD closure. The incidence of heart block may be less than reported with the original device. < Learning objective: Use of Amplatzer® duct occluder is effective in transcatheter perimembranous ventricular septal defect (pmVSD) closure in selected patients. It may be safer than original Amplatzer pmVSD occluder to avoid complete atrioventricular block.> [ABSTRACT FROM AUTHOR]

The introduction of the arterial switch operation has improved the surgical outcome of transposition of the great arteries. However, coronary anomalies such as intramural coronary arteries, single coronary artery, or coronary arteries originating from a single arterial sinus have been reported as independent risk factors for early mortality and late morbidity after an arterial switch operation. We performed an arterial switch operation using a unique technique for translocation of the coronary arteries originating from a single left-side arterial sinus, to prevent coronary artery distortion and subsequent coronary malperfusion. [ABSTRACT FROM AUTHOR]

Background: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history.Case Presentation: A 64-year-old man was diagnosed as having multiple atrial septal defects. He presented with no symptoms of heart failure. His preoperative ECG showed complete right bundle branch block (CRBBB) with a corrected QT interval time of 478 ms. He underwent open-heart surgery to close the defects through median sternotomy access. Three hours after the operation, he developed multiple events of TdP and VFs in the intensive care unit. Cardiopulmonary resuscitation and multiple cardioversions were attempted for his repetitive TdP and VFs. He eventually reverted to sinus rhythm, and intravenous beta-blocker was administered to maintain the sinus rhythm. After this event, his family history was reviewed, and it was confirmed that his daughter and grandson had a medical history of arrhythmia. A genetic test confirmed that he had a missense mutation in CACNA1C, p.K1580 T, which is the cause for type 8.Conclusions: This case highlights the importance of paying attention to other ECG findings in patients with CRBBB, which can mask prolonged QT intervals. [ABSTRACT FROM AUTHOR]

Background Recently, a student died of idiopathic ventricular fibrillation in a school where an automated external defibrillator ( AED) had been installed. The tragedy could not be prevented because the only AED in the school was installed in the teachers' office, far from the school ground where the accident took place. This prompted establishment of a multiple AED system in schools. The aim of this study was to analyze the efficacy of the multiple AED system to prevent sudden death in school-aged children. Methods Assumed accident sites consisted of the school ground, gymnasium, Judo and Kendo hall, swimming pool, and classrooms on the first and the fourth floor. Multiple AED were installed in the teachers' office, gymnasium, some classrooms, and also provided as a portable AED in a rucksack. The time from the accident site to the teachers' office for single AED, and from the accident site to the nearest AED for multiple AED, was calculated. Results The AED retrieval time was significantly shorter in 55 elementary schools and in 29 junior high schools when multiple AED were installed compared with single AED. Except for the classroom on the fourth floor, the number of people who took >120 s to bring the AED to the accident site was lower when multiple AED were installed compared with the single AED. Conclusion Multiple AED provided in appropriate sites can reduce the time to reach the casualty and hence prevent sudden death in school-aged children. [ABSTRACT FROM AUTHOR]

Open heart surgery for infants with low body weight (BW) remains still a challenge. Pulmonary artery banding (PAB) is a useful surgical palliation for small neonates and early infants with excessive pulmonary blood flow who are unable to withstand a heart surgery. This study retrospectively reviewed neonates and infants who underwent PAB to assess the surgical results and the validity of our PAB. We selected 38 acyanotic infants and neonates and divided them into 2 groups: low BW (< 2.5 kg, n = 15, group L) and normal or high BW (≥ 2.5 kg, n = 23, group NH). The average BW at the time of PAB was 2.8 ± 1.1 kg (range, 1.2-5.8 kg), and the average age at the time of PAB was 41.8 ± 44.8 days (range, 2-151 days). Using a 3-mm-wide polyester tape, we tightened the main pulmonary artery to obtain the circumference of (19 mm + 1 mm for each kg of BW). There was no early death but one late death in each group. Postoperative BW continuously increased 1 month after PAB in both groups, although BW was significantly lower in group L than in group NH. Intracardiac repair (ICR) was accomplished in 31 patients (13 in group L and 18 in group NH) at average ages of 1.5 years, while the remaining 5 patients are awaiting ICR. In conclusion, PAB using our formula for the infants even weighing < 2.5 kg has low mortality and is effective as a bridge to ICR. [ABSTRACT FROM AUTHOR]