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Key Clinical Message: Deep femoral artery aneurysms (DFAA) are extremely rare. We treated four DFAAs with different procedures including surgical, endovascular, and hybrid surgery. The best treatment should be selected for each individual case. We report three cases of deep femoral artery aneurysms treated with different techniques. Case 1: A 69‐year‐old man with a huge deep femoral artery aneurysm underwent open reconstruction using a 6 mm expanded polytetrafluoroethylen graft. Case 2: A 67‐year‐old man presented with bilateral deep femoral artery aneurysms. The right‐sided rupture was treated with hybrid embolization, while the left aneurysm was treated by endovascular stent‐grafts deployment. Case 3: A 87‐year‐old man with a large deep femoral artery aneurysm underwent simply surgical aneurysmectomy. As there are many treatment options for deep femoral artery aneurysms, a comprehensive preoperative assessment is essential, encompassing an evaluation of symptoms, anatomy, and comorbidities. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Acute valve thrombosis after bioprosthetic aortic valve replacement even under anticoagulation therapy is extremely rare. Cardiac computed tomography is a powerful imaging tool to detect valve thrombosis, and surgery is necessary in case of unstable hemodynamics. [ABSTRACT FROM AUTHOR]

Recently, several centers have performed total arch replacement using the fenestrated frozen elephant trunk technique for acute Stanford type‐A aortic dissection. However, the long‐term results and need for additional treatment following this procedure are unclear. We report a case of a 54‐year‐old man who underwent endovascular therapy for endoleaks after total arch replacement using the fenestrated frozen elephant trunk technique for acute type‐A aortic dissection with an isolated left vertebral artery. After the surgery, the endoleak was resolved, and the patient was asymptomatic with no neurological deficits. This strategy might be effective in similar cases. When endoleak occur after TAR using the fenestrated frozen elephant trunk technique in patients with rare anatomical subtypes, collaboration with other experts facilitates additional treatment. [ABSTRACT FROM AUTHOR]

Sinus of Valsalva aneurysm is a rare disease characterized by the partial elevation of the aortic root. Few reports are available on the surgical treatment for infants. We report the repair of an extremely rare case of a sinus of Valsalva defect with a ventricular septal defect and right ventricular outflow tract stenosis in an infant. It was not a sinus of Valsalva aneurysm, but it exhibited abnormal partial bulging of the aortic root and forming an aneurysm‐like cavity within the right ventricular myocardium. We performed direct closure of the sinus of Valsalva aneurysm‐like cavities and intracardiac repair in two stages. Three years after total repair, the patient remained healthy and asymptomatic. [ABSTRACT FROM AUTHOR]

Surgical treatment of cardiac fibroma is rare in patients with left ventricular noncompaction (LVNC). Although several case reports regarding cardiac fibroma have been published, resection in a patient with LVNC has not been described. Here, we describe the surgical treatment of left ventricular fibroma in a child with LVNC. We resected a cardiac fibroma in a 10-year-old boy with LVNC to control ventricular arrhythmia. Partial resection with careful tumor dissection was performed to avoid endocardial damage and entering the ventricular cavity. The postoperative course was uneventful, and the patient remains asymptomatic without heart failure or arrhythmia. Surgical excision of cardiac fibroma can be performed safely with excellent results, even in a child with LVNC. [ABSTRACT FROM AUTHOR]

Background: A double‐chambered right ventricle is a rare congenital heart disease where an anomalous structure divides the right ventricle into two cavities. Case: A 78‐year‐old man with dyspnea was referred to our institution for a double‐chambered right ventricle (DCRV) and ventricular septal defect (VSD). Echocardiography showed normal left ventricular function, right ventricular hypertrophy, and mild tricuspid regurgitation. On parasternal short‐axis views, color‐flow Doppler studies showed a mosaic pattern through the stenotic right ventricular outflow tract (RVOT). Cardiac catheterization showed a 122 mmHg pressure gradient between the high‐pressure chamber and the low‐pressure chamber of the RVOT. Computed tomography showed a cap‐like structure consisting of fibrous tissue, with moderate stenosis, which divided the right ventricle into two cavities. The aberrant tendinous chords supporting the anterior leaflet of the tricuspid valve were found inserted into the anterior wall of the RV (Figure 1). Resection of the cap‐like fibrous tissue and abnormal muscle (Figure 2), detachment of aberrant tendinous chords of the tricuspid valve, closure of VSD by direct suture, and tricuspid valve plasty (TVP) were performed. TVP was achieved using an artificial chordae replacement with expanded polytetrafluoroethylene (CV‐5) suture and a 32‐mm Physio Tricuspid annuloplasty ring (Edwards Lifeline). Postoperative echocardiography revealed no RVOT pressure gradient and a normal right ventricular pressure of 21 mmHg. Discussion: In conclusion, we report a rare case of DCRV secondary to a cap‐like structure fibrous tissue with anomalous chordal insertion of a tricuspid valve leaflet. [ABSTRACT FROM AUTHOR]

Supravalvular aortic stenosis as a late complication of transposition of the great arteries is very rare, and only a few cases have been reported. We describe the case of a 14-year-old girl who developed supravalvular aortic stenosis as a late complication of the arterial switch operation for transposition of the great arteries. The narrowed ascending aorta was replaced with a graft. The right pulmonary artery was transected to approach the ascending aorta which adhered severely to the main pulmonary trunk, and we obtained a good operative field. [ABSTRACT FROM AUTHOR]

Despite improvements in bioprosthetic valve function, increased human life-expectancy has led to a growing number of bioprosthetic valve deterioration cases requiring reoperation. We report 2 cases of primary tissue failure of a bioprosthetic valve, which were treated by mitral valve replacement using the valve-on-valve method. The reasons for the reoperations were a severely calcified valve annulus, and severe adhesion of a previous bioprosthetic valve. We removed only the leaflets of the bioprosthetic valve and sutured a Carbomedics OptiForm valve onto the sewing cuff of the previous bioprosthesis. No complications, including major cardiac events, were noted during the follow-up. [ABSTRACT FROM PUBLISHER]

Presents information on a study which examined the effects of an isolated change in load in the renin-angiotensin system on the hypertrophic response to hemodynamic overloading. Methodology; Results and discussion.

Studies whether the cardiac hypertrophy which develops in dogs in response to mitral regurgitation is secondary to change in the rate of protein synthesis or due to decreased rate of protein degradation. Mechanism of myocardial hypertrophy; Materials and methods; Discussion on the results of the study.

Studies the role of renin-angiotensin system (RAS) in regulating cardiac hypertrophy in response to a hemodynamic overload. Examination on the left ventricular hypertrophic response to a pressure overload in mice devoid of the AT1A receptor; Involvement of multiple mechanisms in cardiac hypertrophic process; Putative major effector of the growth response of the RAS.

Presents information pertaining to the pressure-overload hypertrophy which is abated in mice devoid of adenosine triphosphate (AT)1A receptors. Reference to the mechanisms controlling cardiac growth being under intense investigation; Examination of the left ventricular hypertrophic response.

Planning for the acute phase of ischemic stroke in postoperative patients with aortic dissection is difficult from the perspective of concerns about worsening disease related to aortic dissection due to intravenous thrombolytic agents and securing access routes when mechanical thrombectomy is planned. Herein, we report that a 52-year-old man underwent thoracic endovascular aortic repair for acute type B aortic dissection. One year after the procedure, the patient developed a stroke caused by stent graft thrombosis, and computed tomography angiography showed occlusion of the left common carotid artery and left internal carotid artery. Stroke neurologists performed mechanical thrombectomy via a direct approach from the left common carotid artery, and successful recanalization was achieved. Furthermore, ligation of the proximal portion of the left common carotid artery and bypass surgery on the distal portion of the left common carotid artery were performed by cardiovascular surgeons. Although the patient had a postoperative hemorrhagic infarction, he returned to work without a recurrence of stroke after two years of follow-up. A direct carotid artery puncture we performed is an alternative in cases of anatomical difficulty or an unfavorable aortic arch. This case highlights not only the significance of interdisciplinary collaboration between cardiac and neurological specialists but also the impact of training dual-specialty cerebrovascular neurosurgeons on patient outcomes., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Saito et al.)

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH., Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death., Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 ( P  = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P  = .009)., Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Chida-Nagai, Masaki, Maeda, Sasaki, Sato, Muneuchi, Ochiai, Murayama, Tahara, Shiono, Shinozuka, Kono, Machida, Toyooka, Sugimoto, Nakamura, Akagi, Kondo, Kasahara, Kotani, Koizumi, Oda, Harada, Nakajima, Murata, Nagata, Yatsunami, Kobayashi, Matsunaga, Inoue, Yamagishi, Nakagawa, Ohtani, Yamamoto, Ito, Hokosaki, Kuwahara, Masutani, Nomura, Wada, Sawada, Abiko, Takahashi, Ishikawa, Okada, Naitoh, Toda, Ando, Masuzawa, Hoshino, Kawada, Nomura, Ueno, Ohashi, Tachibana, Cao, Ueda, Yanagi, Koide, Mitsushita, Higashi, Minosaki, Hayashi, Okamoto, Kuraishi, Ehara, Ishida, Horigome, Murakami, Takei, Ishii, Harada, Hirata, Maeda, Tatebe, Ota, Hayabuchi, Sakazaki, Sasaki, Hirono, Suzuki, Yasuda, Takeda, Sawai, Miyaji, Kitagawa, Nakai, Kakimoto, Agematsu, Manabe and Saiki.)

Bioprosthetic aortic valves have limited durability. We herein report sudden progression of structural valve deterioration (SVD) and a successful case of emergency transcatheter aortic valve (TAV) implantation for acute decompensated heart failure (ADHF) caused by SVD. A 79-year-old man who had undergone a Bentall operation 11 years prior was diagnosed with ADHF due to suddenly progressive SVD. Emergency TAV implantation in the surgical bioprosthetic valve was selected based on the surgical risk. Ours and previous case reports suggest that SVD can progress suddenly, even after months of stability, and that emergency TAV implantation is effective.

A 46-year-old man presented with sudden onset of chest pain. He was in cardiogenic shock at arrival. Based on the results of ECG and echocardiogram, he was diagnosed with ST-segment elevation myocardial infarction. Point-of-care ultrasonography (POCUS) did not reveal acute aortic dissection (AAD). During an emergency coronary angiography, aortic dissection was detected and computed tomographic angiography (CTA) revealed Stanford type A AAD with a highly compressed true lumen. Because of this form of aortic dissection, the enlarged false lumen could be potentially misidentified as a normal aorta in POCUS. Although POCUS is useful when AAD is suspected, we should not overestimate its findings and lower the threshold for CTA., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND Chromosome 22q11.2 deletion syndrome (22q11.2 DS) currently includes DiGeorge syndrome, conotruncal anomaly face syndrome, and velocardiofacial syndrome. We present the case of a male infant with 22q11.2 DS exhibiting generalized skin rash and dermatopathic lymphadenitis. CASE REPORT The patient was born at 40 weeks of gestation with interruption of aortic arch, ventricular septal defect, and thymic defect. Fluorescence in situ hybridization method performed on buccal smears detected the deletion of 22q11.2. On day of life 33, diffuse erythema appeared on the entire body. A skin biopsy detected vacuolar interface dermatitis with superficial perivascular infiltration. Laboratory examinations revealed eosinophilia and hypocalcemia. Clinically, cutaneous inflammation was correlated with the abnormal immune response in 22q11.2 DS. On day of life 210, the patient died due to sepsis caused by Pseudomonas aeruginosa. An autopsy revealed lymph nodes swellings in the bilateral axillar and subclavicular areas and around the bilateral iliac arteries. Histology of the lymph nodes demonstrated sparse distribution of atrophic germinal centers surrounded by wide zones of proliferating spindle cells, as well as macrophages, Langerhans cells, and interdigitating dendritic cells. Fontana-Masson staining revealed abundant melanin particles in the macrophages. Accordingly, we diagnosed this case as dermatopathic lymphadenitis. Interestingly, CD123 and CD56 double-positive spindle cells also proliferated around the germinal center. CONCLUSIONS This case had an unusual histological feature of dermatopathic lymphadenitis. Considering the wide variety of unusual immune conditions in 22q11.2 DS, the lymph nodes in the systemic skin inflammation may exhibit an extraordinary histology of spindle cells proliferation.